47: Care of the Patient With a Blood or Lymphatic Disorder

The average adult has a circulating blood volume of 5 to 6 L. It is this circulation that allows blood to perform some of its critical functions. Blood transports oxygen and nutrients to cells; vital components of the immune system to injured areas of the body or sites of infection; coagulation factors to prevent blood loss and initiation of clotting; hormones released by endocrine glands to their target tissues; and waste products released by cells to other organs for further processing and/or excretion. Blood maintains a slightly alkaline pH of 7.35 to 7.45. This pH is maintained via the regulation of substances dissolved in blood known as buffers.

Fig. 47.1 Hematocrit (Hct) tubes showing normal blood, anemia, and polycythemia. Note the buffy coat located between the packed red blood cells (RBCs) and the plasma. (A) A normal percentage of RBCs. (B) Anemia (a low percentage of RBCs). (C) Polycythemia (a high percentage of RBCs). *WBCs*, White blood cells.

Components of Blood Relevant to the Hematological System

Erythrocytes or Red Blood Cells

A RBC is known as an erythrocyte in its mature form. Immature RBCs are known as reticulocytes and may be released from the bone marrow in times of stress. Erythrocytes make up the major cellular component circulating in blood. These cells differentiate from stem cells, and as they differentiate, they eventually lose their nucleus and obtain a biconcave disk shape. The biconcave disk shape is vital to their function, as it allows for better maneuverability through capillary beds. Erythrocytes consist of cytoplasm and mainly the protein hemoglobin that contains iron. The presence of hemoglobin allows erythrocytes to perform their essential function of gas exchange. When the hemoglobin in erythrocytes is saturated with oxygen molecules, blood appears a brighter red than when it is carrying carbon dioxide. Therefore, arterial blood is a brighter red in color than venous blood.

The lifespan of an erythrocyte is 120 days. Aged and damaged erythrocytes are removed from circulation by the spleen and liver. The hemoglobin and iron contained within the erythrocyte are mostly recycled to make new erythrocyte cells. The rest of the hemoglobin is broken down into bilirubin and secreted in bile which is then excreted in fecal matter and urine. Iron is lost in fecal matter, urine, and monthly menstrual flow.

Aged and damaged erythrocytes are replaced by the production of new ones from the bone marrow. This means that the bone marrow is actively producing new erythrocytes. The production of erythrocytes is known as erythropoiesis . The bone marrow requires certain essential vitamins, proteins, and minerals to produce healthy mature erythrocytes. When the nutrient needs are not met, the production of erythrocytes is altered, which results in hematological disease, mainly anemias.

Erythrocytes may also be produced from the bone marrow in response to alterations in oxygen saturation levels. As blood filters through the kidney, specialized cells in the kidney sense oxygen levels. When oxygen levels are low, the kidney produces a hormone known as erythropoietin. Erythropoietin is then transported via blood to the bone marrow where it stimulates the bone marrow to increase production of erythrocytes. Healthy mature erythrocytes are produced in response to this stimulation as long as the bone marrow has an available and healthy supply of folic acid, iron, vitamin B₁₂, pyridoxine (vitamin B6), amino acids, etc. In addition, disease within the kidney can result in alterations in sensing oxygen saturation levels or its ability to produce and release erythropoietin. Thus, patients with kidney disease may suffer from anemia known as anemia of chronic kidney disease.

Leukocytes or White Blood Cells

WBCs are known as leukocytes and are the body’s primary defense mechanism against foreign invaders. There are five types of leukocytes, which are divided into two broad categories—granulocytes and agranulocytes. Granulocytes are so named because these cells contain granules inside of them that become apparent when stained in a laboratory with Wright’s stain. Agranulocytes, on the other hand, contain no granules and do not absorb Wright’s stain.

Granulocytes

There are three types of leukocytes that are granulocytes. These are neutrophils, basophils, and eosinophils. Basophils and eosinophils are further discussed in the immune chapter (Chapter 55), as their primary role involves allergic and hypersensitivity reactions. Neutrophils are also called polymorphonuclear neutrophils (PMNs or polys) or segmented neutrophils (segs). Mature neutrophils differentiate from the myeloid stem cell and contain a multi-lobed nucleus that is connected by thin filaments. On microscopic examination, this nucleus appears segmented, thus leading to a name of segmented neutrophils. Immature neutrophils are referred to as band cells, as their nucleus on microscopic examination consists of one elongated nucleus that is not “cut” into multiple lobes. As in the case of reticulocytes, band cell numbers may increase in blood supply during times of stress. An increase in band cells within the blood may be referred to as a shift to the left, as typically band cell numbers are recorded on the left side of the paper.

Segmented neutrophils produced and released from the bone marrow circulate within blood for a few short hours before migrating into body tissues. Here, they surveil for inflammation and possible invasion by bacteria and fungi. Neutrophils are primary defenders and arrive at the site of inflammation within an hour of onset. They ingest pathogens and dispose of dead tissue using phagocytosis. Neutrophils also release lysozyme from their granules, which is an enzyme that destroys the cell wall of bacteria, thereby causing bacterial contents to leak out, resulting in cessation of the bacterial cell cycle and death. Once recruited in the immune response, a neutrophil dies within 1 to 2 days. The bone marrow is constantly producing neutrophils, and their numbers remain stable in a healthy person; however, when challenged by serious infections, the bone marrow can quickly produce and release large numbers of neutrophils into circulation.

Spleen

The spleen is a soft, roughly ovoid, highly vascularized organ located in the left upper quadrant of the abdominal cavity, just below the diaphragm (Fig. 47.3). The spleen is 5 to 6 in (12.7 to 15.2 cm) long and 2 to 3 in (5 to 7.6 cm) wide. It is the largest of the organs in the lymphatic system. The spleen contains two main regions referred to as red pulp and white pulp. The red pulp contains venous sinuses filled with blood and connective tissue that contains red cells and white cells. The white pulp consists of B-cells and T-cells. As blood flows into the spleen, it enters a maze of passages. Within these passages, blood is surveilled for healthy and damaged/dying RBCs. Healthy RBCs continue to flow through the passages while damaged/dying RBCs are taken out of circulation and broken down by macrophages. When breaking down RBCs, hemoglobin and iron are stored by the spleen to be reused by the bone marrow when making more RBCs. In addition to processing and removing damaged RBCs from circulation, the spleen also stores roughly 500 mL of blood, which it will release into circulation in the event of an emergency. The storing of blood is what gives the spleen its deep purple color. The white pulp of the spleen is instrumental in destroying pathogens that enter the spleen via blood, thereby cleaning the blood supply as it flows through the spleen.

Thymus

The thymus gland (see Fig. 47.3) is a specialized lymphoid organ that is soft and roughly triangular in shape. It is located in the mediastinum, anterior and superior to the heart, posterior to the sternum. It houses immature T-cells known as thymocytes and contains specialized epithelial cells that help thymocytes mature into T-cells. These T-cells then migrate to the spleen and lymph nodes where they play a critical role in immune defense. The thymus gland plays the largest role in immunity during neonatal and pre-adolescent years. In pre-teen years, it starts to atrophy in size and activity. In adults, the thymus gland is mostly replaced by fatty tissue, though the maturation of T-cells continues throughout life.

Laboratory and Diagnostic Tests

Complete blood count

The complete blood count (CBC) is an important screening tool. It is used to confirm and investigate for many disease processes. The CBC detects many disorders of the hematologic system and provides data for diagnosing and evaluating disorders in other body systems. A CBC can be ordered as a CBC or a CBC with differential. Most of the times it is ordered as a CBC with differential, which gives the values of the five types of WBCs separately. Further details of this important screening tool are displayed in Table 47.1. A CBC includes red cells, white cells, and platelets; hematocrit (Hct) and hemoglobin (Hgb) levels; erythrocyte indexes; differential WBC count; and examination of the peripheral blood cells. Prepare the patient by explaining that a blood sample will be taken from a vein. The hand and arm (usually the antecubital fossa) are used commonly as sites for blood collection.

Table 47.1

Diagnostic Blood Studies
Blood Test	Normal Values			Description
White Blood Cells
White Blood Cells (WBCs) or Leukocytes	5000–10,000 cells/mm³			Actual cell count
Leukocytopenia—total WBC cells count is lower than 5000 cells/mm³ Present in chemotherapy, radiation, aplastic anemia		Low High	Leukocytosis—total WBC count is higher than 10,000 cells/mm³ Present in infections caused by a variety of pathogens, inflammatory disorders, trauma, and leukemia
Neutrophils	60%–70% 3000–7000 cells/mm³ ^a (60% × 5000) to (70% × 10,000)			Cell count based on normal percentage values for neutrophils; calculated by taking 60%–70% of WBC high and low range
Neutropenia—percentage of neutrophils present among total WBC count is less than 60% Present in patients exposed to chemotherapy and radiation, agranulocytosis, and autoimmune diseases		Low High	Neutrophilia—percentage of neutrophils present among total WBC count is higher than 70% Present in burns, crushing injuries, diabetic ketoacidosis, infections
Eosinophils	1%–4% 50–400 cells/mm³ ^a (1% × 5000) to (4% × 10,000)			Cell count based on normal percentage values for eosinophils; calculated by taking 1%-4% of WBC high and low range
A concern if low along with other white blood cells, with alcohol intoxication, or excessive cortisol production		Low High	Increased in allergic reactions and with parasitic disorders
Basophils	0.5%–1% 25–100 cells/mm³ ^a (0.5% × 5000) to (1% × 10,000)			Cell count based on normal percentage values for basophils; calculated by taking 0.5%-1% of WBC high and low range
May indicate a severe allergic reaction		Low High	Can occur due to chronic inflammation
Lymphocytes	20%–40% 1000–4000 cells/mm³ ^a (20% × 5000) to (40% × 10,000)			Cell count based on normal percentage values for lymphocytes; calculated by taking 20%-40% of WBC high and low range
Lymphocytopenia—percentage of lymphocytes present among total WBC count is lower than 20% Present in AIDS, SLE, and Hodgkin lymphoma.		Low High	Lymphocytosis—percentage of lymphocytes present among total WBC count is higher than 40% Present in infectious mononucleosis, measles, infectious hepatitis, and lymphocytic leukemia.
Monocytes	2%–6% 100–600 cells/mm³ ^a (2% × 5000) to (6% × 10,000)			Cell count based on normal percentage values for monocytes; calculated by taking 2%-6% of WBC high and low range
Monocytopenia—percentage of monocytes present among total WBC count is lower than 2% Present with aplastic anemia, hairy leukoplakia, thermal injuries, and treatment with corticosteroids		Low High	Monocytosis—percentage of monocytes present among total WBC count is higher than 6% Present with recovery phase from bacterial infections and chronic inflammatory conditions
Platelets
Platelets or thrombocytes	150,000–400,000 cells/mm³			Actual cell count
Thrombocytopenia—number of circulating platelets is less than 150,000 cells/mm³. Present in thrombocytopenia and aplastic anemia.		Low High	Thrombocytosis—number of circulating platelets is more than 400,000 cells/mm^3. Present in granulocytic leukemia; bone marrow suppression, such as with chemotherapy or radiation therapy
Table Continued

Blood Test	Normal Values			Description
Red Blood Cells
Red Blood Cells or erythrocytes	Males 4.7–6.1 million cells/μL Females 4.2–5.4 million cells/μL			Actual cell count
Erythrocytopenia or erythropenia—number of circulating red blood cells is less than 4.7 (Males) or 4.2 (Females) million cells/uL Present in anemia due to bleeding, iron deficiency anemia, megaloblastic anemia, sick cell, and hemophilia; kidney disease or damage Decreased number of red blood cells directly impacts the amount of cells present that can carry oxygen and therefore results in reduced oxygen carrying capacity. Since there are fewer red blood cells, the hemoglobin count will also be decreased.		Low High	Erythrocytosis—number of circulating red blood cells is more than 6.1 (Males) or 5.4 (Females) million cells/uL Present in polycythemia vera Causes blood to be more viscous which can lead to clotting
Hemoglobin	Males: 13.5–17.5 g/dL Females: 12–15.5 g/dL			Actual count of the amount of hemoglobin in grams per deciliter of blood
Oxygen-carrying capacity in the blood is directly impacted, as oxygen is carried in blood by binding to hemoglobin Present in iron deficiency anemia, folate deficiency anemia, vitamin B 12 deficiency anemia, blood loss such as hemophilia, sickle cell anemia		Low High	Usually high because the red blood cell count is also higher. This indicates a compensatory mechanism. Present in thalassemia, renal disease, dehydration, pulmonary problems, and polycythemia vera
Hematocrit	Males: 42%–52% Females: 35%–47%			Volume of red blood cells divided by the total volume of the sample multiplied by 100%
Low hematocrit indicative of fluid overload, blood loss, leukemia, iron deficiency anemia, vitamin B₁₂ deficiency anemia, sickle cells anemia, hemophilia		Low High	High hematocrit indicative of dehydration, polycythemia vera, pulmonary disease, renal disease
Mean Corpuscular Volume ^b	80–100 fL (femtoliters)			Measure of the average size and volume of a red blood cell
Average size and volume of a red blood cell are smaller than normal, indicating microcytic anemia; conditions classified as microcytic anemia include iron deficiency anemia, sideroblastic anemia, and thalassemia’s (Maner & Mosavi, 2020).		Low High	Average size and volume of a red blood cell are larger than normal, indicating macrocytic anemia; conditions classified as macrocytic anemia include megaloblastic anemia (folate deficiency, vitamin B₁₂ deficiency, orotic aciduria) and non-megaloblastic anemia due to hepatic insufficiency, chronic alcoholism, and Diamond Blackfan anemia (Maner & Mosavi, 2020)
Red Cell Distribution Width	Males: 12.2%–16.1% Females: 11.8% -14.5%			Measures the variance of the volume and size of red blood cells; the difference in volume and size from the biggest to the smallest red blood cell
Indicates all red blood cells are about the same volume and size Not associated with any hematological disorder (May, Marques, Reddy, et al., 2019).		Low High	Indicates red blood cells vary in size from small to big at a higher percentage than normal Usually caused by nutritional deficiencies such as Vitamin B₁₂, folate, and iron; hallmark of iron deficiency anemia in the early stages of the disease; can also indicate acute hemorrhage or hemolysis
Table Continued

Blood Test	Normal Values			Description
Mean Corpuscular Hemoglobin (MCH) ^b	27.5–33.2 pg (picograms)			Average mass of hemoglobin that is in each red blood cell, which is determined by iron
As the mass of hemoglobin depends on iron content, low MCH is indicative of iron deficiency anemia		Low High	Occurs with macrocytic anemias, as the cells are too big, and so the average mass of hemoglobin increases Occurs with folic acid deficiency or vitamin in B₁₂ deficiency
Mean Corpuscular Hemoglobin Concentration (MCHC) ^b	33.4–35.5 g/dL			Average weight of the hemoglobin in a volume of red blood cells
Indicative of iron deficiency anemia		Low High	Indicative of sickle cell anemia

Peripheral blood smear

A peripheral blood smear, along with the differential CBC, allows examination of the size, shape, and structure of individual blood cells and platelets. This information is useful in differentiating various forms of anemias and dyscrasias (any abnormal physiologic condition, especially of the blood). All three hematologic cell lines (RBCs, WBCs, and platelets) can be examined. When adequately prepared and examined microscopically by an experienced technologist, a peripheral blood smear is the most informative of all hematologic tests.

Schilling test and megaloblastic anemia profile

The Schilling test was developed by Dr. Robert F. Schilling to aid in diagnosing a vitamin B₁₂ deficiency. Vitamin B₁₂ deficiency can occur due to deficiencies in diet, lack of intrinsic factor, or due to lack of absorption. The Schilling test aids in pinpointing the cause of the deficiency. In stage 1 of the test, patients suspected to have vitamin B₁₂ deficiency are given radio-cobalt-labeled vitamin B₁₂ orally and are put on a 24-hour urine collection. Patients with diet deficiencies in vitamin B₁₂ will have a normal result of 8% to 40% of radioactive cobalt vitamin B₁₂ excretion in urine within 24 hours. Patients with either a lack of intrinsic factor or absorption issues will have low levels of radioactive-labeled vitamin B₁₂ in the excreted 24-hour urine collection. In stage 2 of the test, patients are again given radiolabeled vitamin B₁₂ orally along with an oral dose of intrinsic factor and are again put on a 24-hour urine collection. If the 24-hour urine collection shows a normal result of 8% to 40% of radioactive vitamin B₁₂ excretion, then the patient is lacking intrinsic factor and has pernicious anemia. If, however, the 24-hour urine collection shows an abnormal level of excreted radiolabeled vitamin B₁₂, the patient has absorption issues in the terminal ileum, and other disease processes need to be investigated to determine the cause of vitamin B₁₂ deficiency (Ramphul & Mejias, 2019). Despite the diagnostic value of this, it is no longer used to diagnose the etiology of vitamin B₁₂ deficiency due to the decreased availability of radiolabeled cobalt and as advances in science have allowed for other laboratory studies to be performed. Today, vitamin B₁₂ deficiency is diagnosed using a CBC, serum vitamin B₁₂ levels, and methylmalonic acid and homocysteine levels (both of which are converted to other substances only in the presence of vitamin B₁₂). Together, these four tests are known as the megaloblastic anemia profile.

Radiologic studies

Radiologic studies of the hematologic system involve primarily the use of computed tomography (CT) or magnetic resonance imaging (MRI) for evaluating the spleen, liver, and lymph nodes. In the past, lymphangiography (i.e., x-ray analysis of lymphatic vessels and nodes) with contrast dye was a common procedure for evaluating lymph nodes deep inside the body.

Bone marrow aspiration or biopsy

Bone marrow aspiration and biopsy can help establish a diagnosis when there is a suspected hematologic issue. These are invasive procedures, therefore they are used judiciously. Bone marrow aspiration and biopsy are used to investigate anemias, penia or cytosis in WBCs or platelets, cancers within the bone marrow, and fevers of unknown origin. The most common site for this procedure is the posterior iliac crest due to a lack of vital organs, major blood vessels, and nerves in this area. In some cases the sternum can be used as well, though when used, it is mostly to perform an aspiration as opposed to a biopsy.

Normal bone marrow is soft and semifluid and can be removed by aspiration through a specialized needle device. Bone marrow aspiration is most commonly performed in people with marked anemia, neutropenia (decreased number of certain WBCs), acute leukemia, and thrombocytopenia (decreased number of platelets). The aspirate is examined for cell types, numbers, and degree of maturation. Because the aspirate is a sample of cells, it may not provide the degree of detail that can be gained from a biopsy, therefore it is possible that a biopsy may be necessary after an aspiration procedure. In a bone marrow aspiration, 5 mL of blood and marrow is aspirated. If a biopsy is needed, it is best performed after an aspiration in a slightly different location. A bone marrow biopsy garners a core sample that better represents the cells in the marrow than an aspiration procedure and has a greater diagnostic value. Patients undergoing either of these procedures need an explanation of the procedure and a signed informed consent. Patients undergoing an aspiration or biopsy procedure experience a pressure sensation as the needle is advanced. Aspiration causes brief but sharp pain, while biopsy causes a pressure sensation but no pain. Post procedure, complications include a risk of bleeding and an ache at the site of the biopsy for 1 to 2 days. The needle insertion site is usually covered with a small sterile dressing.

Hematological Therapies

Splenectomy

A splenectomy is the surgical removal of the spleen and a treatment option for patients with various hematological disorders. Hypersplenism, overactive spleen, is a condition where the spleen destroys excessive numbers of RBCs and platelets instead of just destroying damaged or dead cells, thus resulting in anemia and clotting disorders. The removal of the spleen ceases this excessive destruction, thus causing a rebound in the numbers of circulating RBCs and platelets. A splenectomy is a treatment of choice for patients with immune thrombocytopenia purpura (ITP), cancers such as Hodgkin and non-Hodgkin lymphoma, genetic conditions such as sickle cell disease and thalassemia, splenomegaly, presence of tumors, abdominal injury that results in trauma and subsequent bleeding to the spleen, and in incidences of splenic infarction or aneurysm.

A splenectomy can be performed either open or laparoscopically. Open splenectomy is preferred in gross enlargement of the spleen and if the patient has experienced abdominal trauma. In most cases, a splenectomy is performed laparoscopically. Preoperative assessment includes the status of major organ systems such as cardiovascular, respiratory, and gastrointestinal (GI). Drugs such as aspirin, blood thinners, and anti-inflammatory medications must be stopped at least 1 week prior to surgery. Time permitting, patients are also advised to receive the pneumococcal pneumonia vaccine, as post splenectomy this infection poses the greatest risk, especially in children. The Centers for Disease Control and Prevention (CDC) also recommends scheduled vaccinations for influenza, Haemophilus influenza type B (Hib), meningococcal, HPV, MMR, varicella, and Tdap (tetanus, diphtheria, and pertussis) (CDC, 2016). Postoperative assessment includes comparison of major organ system functions with baseline data recorded during the preoperative assessment. The patient is also monitored for signs and symptoms of complications such as infection, hemorrhage, blood clots, paralytic ileus, and injury to adjacent organ structures. Post splenectomy, patients are at a greater risk of getting serious infections, and it takes them longer to recover from injuries or illness. Patients are taught to notify the provider at the first sign of an infection.

Hematopoietic stem cell transplant

A hematopoietic stem cell transplant (HSCT), more commonly known as a bone marrow transplant, may potentially be a lifesaving procedure for patients with diseases such as leukemia, aplastic anemia, myelodysplastic syndrome, thalassemia, and many more. The process starts by first identifying the source of the stem cells to be transplanted. Autologous HSCT occurs when the bone marrow to be transplanted is taken from the patient afflicted with the disease. Allogenic HSCT occurs when a patient is given stem cells from a donor. Patients suffering from diseases that afflict the bones and bone marrow are generally given allogenic HSCT donations rather than undergoing an autologous transplant.

Allogenic hematopoietic stem cell transplant

Prior to stem cell transplant, a human lymphocyte antigen (HLA) donor match is identified. Stem cells are harvested from the allogenic donor by either conducting a bone marrow harvest or using peripheral blood stem cell (PBSC) harvesting technique. In a bone marrow harvest, stem cells are removed from the pelvic bones of the donor under general anesthesia. A surgeon harvests stem cells by inserting a large needle in multiple places in the pelvic bone and aspirating stem cells. Roughly 1 L of stem cells are harvested using this technique, which takes about 2 hours. Complications experienced by the donor may include bleeding, infection, and nerve damage. PBSC harvesting is a procedure that has been in use for about 10 years and is an effective means of collecting stem cells as a small quantity of stems cells circulate in blood. Donors may or may not be administered cytokines prior to PBSC harvest. Two cytokines (filgrastim and sargramostim) are FDA approved for PBSC harvest and cause an increase in the number of circulating stem cells in the blood by 100-fold. Once peripheral venous access is secured, the donor blood flows from the venous catheter into an apheresis machine where stem cells are separated from the rest of the donor’s blood and the donor’s blood minus stem cells is returned to the donor’s circulatory system. Stem cells are recognized and differentiated from other cells in blood, as they contain a special antigen on their cell surface known as CD34+ antigen. A donor may undergo multiple harvest sessions with the goal of collecting 2 million stem cells/kg of patient weight (Sheth, Jain, Gore, et al., 2020).

Autologous hematopoietic stem cell transplant

Autologous donation is a choice for patients who do not have a suitable allogenic HSCT donor and/or for those with healthy bone marrow but requiring bone marrow ablative chemotherapy prior to transplantation. Autologous HSCT is the preferred donor type for patients with myeloma, non-Hodgkin lymphoma (Sheth, Jain, Gore, et al., 2020), Hodgkin lymphoma, and plasma cell disorders (Mayo Clinic, 2019). Patients receiving an autologous HSCT undergo a PBSC procedure to harvest stem cells. PBSC procedure is preferred over standard bone marrow harvest technique, as it is easier to access and as blood cell counts recover quicker. Patients are given a stem cell mobilizer (cytokines—filgrastim and sargramostim) that increases the amount of circulating stem cells in the blood supply 100-fold. After harvesting the stem cells from the blood via apheresis, stems cells are frozen and the patient is readied for the next step in a HSCT.

Allogenic versus autologous

Donor type used has advantages and disadvantages. Allogenic stem cell transplant is advantageous to the patient, as the stem cells are foreign to the patient’s body, and this allows for cancer cells to be tagged as foreign and destroyed more easily. The ability of stem cells from the donor to more effectively kill a patient’s cancer cells is called the graft-versus-tumor effect. The disadvantage of using an allogenic donor is that the donor’s stem cells may recognize normal cells in the patient as foreign and destroy them as well. In this case, the patient would have graft-versus-host disease (GVHD). GVHD is a complication of allogenic stem cell transplant that can be mild, moderate, or severe. The occurrence and severity of GVHD can be controlled by prescribing immunosuppressive medications. Another disadvantage of using an allogenic donor is the possibility of graft failure. When allogenic donor stem cells are infused into a patient, the stem cells need to migrate to the bone marrow and start producing RBCs, WBCs, and platelets. Successful production of cell lines means that engraftment has occurred, while failure to produce cell lines means the stem cell transplant resulted in graft failure. Allogenic stem cell transplants have the disadvantage of possibly resulting in graft failure, whereas graft failure is extremely rare in an autologous donation. Autologous stem cell transplant also carries the advantage of no GVHD but has the disadvantage of being less effective at killing cancer cells, as the transplanted cells are from the patient. Therefore, the graft-versus-tumor effect may be reduced.

Transplanting stem cells

Patients, whether they are receiving an allogenic or autologous HSCT, undergo either myeloablative or nonmyeloablative chemotherapy and/or radiation. In myeloablative chemotherapy, the patient receives high doses of chemotherapy with the aim of eradicating the existing bone marrow and any malignant cells. In nonmyeloablative chemotherapy, the aim is to destroy malignant cells without completely eradicating all the stem cells in the bone marrow. After administration of chemotherapy, patients are placed in neutropenic precautions and watched closely for signs and symptoms of infection as the cells capable of fighting off infections have been reduced greatly or destroyed. The time frame between chemotherapy/radiation and stem cell transplant depends on the patient’s ability to rest and recover from the ablative therapy. Once recover occurs, stem cells are transplanted via a central venous catheter. Patients may experience side effects from chemicals used to preserve the stem cells and from the effects of an allogenic transplant.

Blood Transfusions

According to the American National Red Cross (2020), less than 38% of the United States population is eligible to donate blood and platelets, and one donation can save potentially three lives. Blood transfusions are conducted every 2 seconds in the United States for a multitude of reasons including surgeries, trauma, GI bleeding, sickle cell disease, hemophilia, and anemias.

ABO blood group

The process of blood transfusions starts with blood typing (see Fig. 47.4). Blood types or groups are inherited from parents. There are four major blood types: type A, type B, type AB, and type O, known collectively as the ABO blood group system. Type A blood contains A antigen on their cell surface, type B blood contains B antigen on their cell surface, type AB blood contains both A and B antigens on their cell surface, and type O blood contains no antigens on their cell surface. When conducting blood typing, a sample of the patient’s blood is mixed with antibody A and antibody B. Type A blood will clump together when exposed to antibody A and have no reaction when exposed to antibody B. Type B blood will clump together when exposed to antibody B and have no reaction to antibody A. Type AB blood will clump together when exposed to both antibody A and B. Type O blood will not clump together when exposed to either antibody A or antibody B.

Patients with type O blood do not have A or B antigens on their cell surface and therefore contain anti-A and anti-B antibodies. They can only be given type O blood in a transfusion, but because they do not have antigens A or B, type O blood can be transfused to patients with type A, type B, and type AB blood. For this reason, type O blood is known as the universal donor. Patient’s with type A blood have A antigens on their cell surface but also contain anti-B antibodies. Therefore, a patient with type A blood can be given type A or O blood, and giving type B blood would cause a blood transfusion reaction. Patients with type B blood have B antigens on their cell surface but also contain anti-A antibodies. Transfusion of type A blood to a person with type B blood would cause the anti-A antibodies to start killing the transfused blood cells, resulting in a transfusion reaction. Patients with type B blood can be transfused with type B or O blood. Patients with type AB blood contains both A and B antigens and have no antibodies for either type of blood. These patients can be transfused type A blood, type B blood, and type O blood. For this reason, type AB blood is known as the universal recipient.

Blood typing is extremely important, as transfusion of incorrect blood types can cause hemolysis (rupturing of RBCs). Hemolysis occurs when the antibodies in the patient receiving the blood attach to the antigens on the donor’s cell surface, causing RBCs to rupture, release cell contents into the blood supply, and die. Symptoms include fever, chills, hypotension, bronchospasm, and low back pain. The blood transfusion must be stopped immediately. There is no rescue medication for this reaction, only prevention through meticulous attention to detail on the part of all health care personnel. Blood transfusions can also cause allergic reactions that are triggered due to plasma proteins within the blood that is being transfused. In an allergic reaction, patients may experience hives (urticaria), flushing, and itching. If this occurs, stop the blood transfusion and resume it again if treatment with antihistamines is successful. Patients who have had multiple transfusions in the past and have a history of known allergic reactions can be given antihistamines prior to the transfusion.

Rh factor

Besides ABO blood grouping, blood typing also determines the Rh factor. The Rh- antigen, sometimes known as Rh (D), is also found in the cell membranes of RBCs. Patients that have the Rh factor are said to be Rh positive while patients who do not have the Rh factor are said to be Rh negative. Similar to ABO typing, Rh factor is determined by exposing a sample of the patient’s blood to Rh antibodies. If the Rh antigen is present, the blood cells will clump and the patient will be recorded as being Rh positive. Patients who are Rh+ may receive blood from a patient that is Rh+ or Rh-, but Rh- patients can only receive Rh- blood. Unlike in ABO blood type, Rh+ antibodies develop in people who are Rh- only on exposure. Subsequent exposures of Rh+ blood in people who are Rh- will cause an antibody reaction and thus a transfusion reaction. The transfusion reaction that occurs when Rh- recipient is exposed to Rh+ blood is less severe than ABO transfusion reactions but not without consequence. Rh incompatibility is seen most commonly in pregnancy. Fortunately, this incompatibility can be prevented. The first step in preventing hemolytic disease of the newborn (HDN) is to find out the Rh types of the expectant parents. If the mother is Rh negative and the father is Rh positive, the baby is at risk for developing HDN. The next step is to test the mother’s serum to make sure she does not already have anti-Rh (also called anti-D) antibodies from a previous pregnancy or transfusion. Finally, the Rh-negative mother is given an injection of Rh immune globulin (RhIg) at 27 to 28 weeks of gestation, and again after delivery, if the baby is Rh positive. The RhIg attaches to any Rh-positive cells from the baby in the mother’s bloodstream, preventing them from triggering anti-D antibody production in the mother. An Rh-negative woman also should receive RhIg after a miscarriage, abortion, or ectopic pregnancy.

Disorders of The Hematologic System

The hematologic and lymphatic systems include the blood and the organs of blood production, the bone marrow, and lymphatic tissue. Disorders of blood production, bone marrow, or lymphatic tissues affect all body systems. Disturbances in this delicate balance can produce life-threatening signs and symptoms, severe pain, and incapacitation.

Disorders Associated With Erythrocytes

Anemia

Anemia is a condition in which there is a below-normal amount of RBCs, causing a decrease in Hgb and Hct. These decreases directly impact all body systems as oxygen carrying capacity is reduced. Anemias can occur due to a deficiency in the production of erythrocytes, destruction of the erythrocytes, or due to bleeding. Impaired production of RBCs can occur due to bone marrow depression, bone marrow disease, or nutritional deficiencies, acting alone or in combination. Bone marrow depression and disease may be of shorter relative duration when occurring with cancer treatments or may be longer depending on disease process. Likewise, nutritional-related anemias can give rise to the onset of long-term anemias. Destruction of erythrocytes may occur due to mismatched blood transfusions and diseases such as sickle cell anemia and thalassemia. It is also possible for destruction of erythrocytes to occur due to autoimmune processes as is the case with autoimmune hemolytic anemia. Another cause of anemias is bleeding, which can occur from the GI tract (peptic ulcers, diverticulosis, ulcerative colitis, colon cancer), due to prolonged menstrual periods, during surgeries, and traumatic events.

Anemias can be classified as chronic or acute. Chronic anemias occur when there is a gradual decline in RBCs, Hct, and Hgb. The gradual decrease allows for the body’s compensatory mechanisms to take effect. Thus, patients with chronic anemias may be asymptomatic or show few symptoms on exertion such as tachycardia and fatigue. Acute anemias cause a rapid decline in RBCs, Hct, and Hgb. This rapid decline does not allow for the body’s compensatory mechanisms to take effect, causing symptoms in patients. Normally, healthy patients experiencing acute anemias can lose up to 20% blood volume without experiencing symptoms, as their body will compensate via reflex vasospasm and redistribution of blood flow to vital organs (Adler & Tambe, 2020).

Clinical Manifestations

Clinical manifestations for anemias are dependent on patient health status, patient activity level, and whether the anemia experienced is acute or chronic. Patients with chronic anemias may show no symptoms and the anemia may be discovered incidentally on routine labs. Some common symptoms experienced in anemias include general malaise, heart palpitations, shortness of breath on exertion, dizziness, and lightheadedness. Patients may also notice that their extremities are colder than before the onset of anemia.

Symptoms of acute anemias will also depend on patient condition prior to the onset of the anemia. Patients who are active and have no comorbid conditions may not manifest symptoms until after 20% blood loss. Symptoms experienced include hypotension, respiratory distress, changes in mental status, chest pain, and cold and clammy extremities. Without correction, patients experience hypovolemic shock, myocardial infarction (MI), stroke, and death.

Assessment

Subjective data commonly include complaints of weakness, dyspnea, fatigue, and vertigo. Anorexia and dyspepsia may accompany headache and insomnia, but the patient generally does not link these complaints to the condition unless questioned. In older adult patients with impaired cardiopulmonary reserves, be alert to complaints of chest pain, dyspnea on exertion, palpitations, and dizziness.

Collection of objective data includes observing signs of bleeding or shock (hypovolemic anemia). Laboratory values show a low RBC count along with low Hct and Hgb levels. Skin and mucous membranes are pale, and cardiac symptoms are related to anemia.

Diagnostic Tests

The CBC of a patient with anemia typically shows a low RBC count and below-normal Hgb and Hct levels. Serum iron, total iron-binding capacity, and serum ferritin levels also may be below normal. Serum folate (also called folic acid) may be measured if certain types of anemia are suspected. The reticulocyte (i.e., immature RBC) count may be increased because of the presence of high numbers of immature RBCs. A bone marrow study shows a deviation from normal findings. Peripheral blood smears enable identification of abnormalities in the shape and color of blood cells. A megaloblastic anemia profile reveals decreased levels of vitamin B₁₂.

Medical Management

Intervention depends on the cause. Correction of the disease process may correct or lessen the anemic condition. Transfusion is appropriate for blood loss; iron, folate, or vitamin B₁₂ are replaced if these are deficient. Treatment is often specific to the particular anemia (see the Cultural Considerations box).

Cultural Considerations

Jehovah’s Witness Opposition to Blood Transfusion

The nurse providing culturally appropriate nursing interventions to a Jehovah’s Witness has many factors to consider. It should be noted that individuals of this religion may vary in their choice. It is important for the nurse to determine the preference for every patient. Jehovah’s Witnesses, typically, are opposed to homologous blood transfusion (blood obtained from a blood bank or through donations). This is based on religious beliefs and not fear of potential risks of receiving a transfusion. Some Jehovah’s Witnesses may agree to certain types of autologous blood transfusions (sometimes called autotransfusions). One type of autologous transfusion that may be acceptable is blood retrieved through induced hemodilution at the start of surgery. This blood conservation technique involves removing blood from a patient—either immediately before or shortly after induction of anesthesia—while simultaneously replacing the blood with an equivalent volume of a blood volume expander (e.g., crystalloid [sodium chloride] and/or colloid). The withdrawn blood is anticoagulated and maintained at room temperature in the operating room for up to 8 hours. It is reinfused into the patient as needed during or after the surgical procedure (JPAC, 2020).

The consensus of the US Supreme Court has been that an adult—that is, a person who has reached the age of majority—has the right to refuse treatment but does not have the right to withhold a potentially life-saving treatment from a minor child.

Some Jehovah’s Witnesses allow the use of certain blood volume expanders (colloids). Many Jehovah’s Witnesses carry a document called an Advance Decision Document with the types of blood volume expanders permitted. Ask the patient for this card or, if the patient is unconscious, examine the patient’s personal belongings to find this extremely important card (JPAC, 2020).

Nursing Interventions and Patient Teaching

Patient problems and interventions for the patient with anemia include but are not limited to the following:

Patient Problem	Nursing Interventions
Compromised Blood Flow to Tissue (cardiovascular), related to reduction of cellular components necessary for delivery of oxygen to the cells	Monitor changes in vital signs and change in LOC Monitor for cardiac rhythms Monitor Hgb, Hct, and RBCs Assess baseline arterial blood gases and electrolytes Note presence and degree of dyspnea, cyanosis Administer blood products as ordered Monitor for blood transfusion reactions
Insufficient Oxygenation, related to deficient: • RBCs • Hgb • Hct	Evaluate ability to manage activities of daily living (ADLs), related to oxygen decrease Assess activity tolerance, dyspnea, heart rate, oxygen saturation, nail beds for cyanosis Observe for cyanosis, hypoxia, and hypercapnia Maintain bed rest as necessary and provide range-of-motion (ROM) exercise Monitor oxygen saturations frequently by pulse oximetry Administer oxygen as ordered Explain activity–oxygen deficit relationship
Inability to Tolerate Activity, related to: • Tissue hypoxia • Dyspnea	Plan care to conserve energy after periods of activity Encourage the patient to limit visitors, phone calls, and unnecessary interruptions to conserve energy Assist the patient with self-care activities as needed Place articles within easy reach of the patient to reduce physiologic demands on the body Administer oxygen as ordered to relieve dyspnea Monitor Hgb and Hct levels

Tailor patient education to the individual’s condition and needs.

Hypoproliferative anemias

A hypoproliferative anemia occurs when the bone marrow fails to produce an adequate number of RBCs. RBC production requires healthy and functioning bone marrow and a supply of nutrients. Anemias that fall into this category include aplastic anemia, iron deficiency anemia, and megaloblastic anemia.

Aplastic Anemia

Etiology and pathophysiology

Aplastic anemia is a rare disorder that is more common in teens, young adults, and older adults, though it can occur at any age. The incidence of aplastic anemia is low, affecting approximately 0.7 to 4.1 per million population each year, and is two to three times more prevalent in Asia than North America and Europe (Zhu, Gao, Hu, et al., 2020). It can occur as an inherited disorder, but it is mostly acquired. The acquired forms of aplastic anemia can occur due to any number of reasons including exposure to toxins, medications, radiation, hepatitis, HIV, Epstein-Barr virus, and pregnancy (Medline Plus, 2018). However, the majority of cases are idiopathic (unknown cause) and thought to occur as an autoimmune condition where the body’s T-cells attack stem cells in the bone marrow, causing damage (Zhu, Gao, Hu, et al., 2020). In aplastic anemia, stem cells in the bone marrow are damaged or destroyed. This causes either a reduction or cessation in the production of erythrocytes, thrombocytes, and leukocytes, a condition known as pancytopenia.

Clinical manifestations

The signs and symptoms of aplastic anemia may have an acute onset or may develop slowly over several weeks to months. These signs and symptoms are directly related to the decreased levels of erythrocytes, thrombocytes, and leukocytes in circulation. Decreased erythrocyte levels cause a concomitant decrease in hemoglobin levels, thus decreasing oxygen carrying capacity. Patients experience the symptoms of anemia such as fatigue, shortness of breath, dizziness, headaches, cold hands and feet, and pallor. A reduction in leukocytes within the circulation results in more frequent and recurrent infections causing symptoms such as fevers, rashes, and flu-like symptoms. Thrombocytopenia results in petechiae, easy bruising or bleeding including from the nose and gums, and blood in the stool. The smallest injury may cause prolonged bleeding to occur.

Assessment

Subjective data include a detailed interview and history relating to chemical exposures, medications the patient may be taking, incidences of infections, and potential exposure to radiation.

Collection of objective data includes monitoring the patient for pallor, shortness of breath, fatigue, tachycardia, chest pain, cold extremities, signs of infection, and bleeding tendencies.

Diagnostic tests

The diagnosis of aplastic anemia begins with a CBC that reveals pancytopenia. A definitive diagnosis can be made with a bone marrow aspiration and biopsy that will show either a hypoplastic or aplastic bone marrow replaced with fat. Post bone marrow studies, aplastic anemia is staged based on the number of cells present into either moderate, severe, or very severe (Johns Hopkins Medicine, n.d.). Other tests conducted include viral studies, liver and kidney studies, levels of vitamin B₁₂ and folic acid, CT, and ultrasound scans. These tests help clinicians navigate through potential causes of aplastic anemia, including perhaps an idiopathic cause.

Medical management

When medications such as cimetidine, anticonvulsants, sulfonamides, or chloramphenicol are identified as the cause of aplastic anemia, the patient is treated by immediately stopping these medications. Bone marrow suppression is expected with certain antineoplastic medications or radiation therapy. In these cases, patients are treated with drugs known as colony-stimulating factors. Filgrastim, pegfilgrastim, and sargramostim stimulate the bone marrow to produce more WBCs; epoetin alfa stimulates stem cells in the bone marrow to produce RBCs; and eltrombopag stimulates production of platelets.

Patients diagnosed with moderate aplastic anemia are generally not treated and monitored via symptoms and lab values. Patients with severe or very severe aplastic anemia, without a HLA-matched sibling donor, and over the age of 35 are treated with immunosuppressive medications (Zhu, Gao, Hu, et al., 2020). Common immunosuppressive therapies used include anti-thymocyte globulin (which kills T-cells) and cyclosporine (which inhibits T-cell proliferation) (Shetty, Narendra, Adiraju, et al., 2016). Patients experiencing complications such as petechiae and bleeding are treated with platelet transfusions, and those experiencing symptoms of anemia due to low RBC levels are transfused packed red blood cells (PRBCs). Multiple blood transfusions carry the complication of iron overload that requires chelation therapy or the possibility of the patient developing antibodies to the transfused blood. Infections are treated and prevented with the use of antibiotics and antivirals.

Patients with severe or very severe aplastic anemia, with a HLA-matched sibling donor, and under the age of 35 are treated with a HSCT (discussed earlier) (Zhu, Gao, Hu, et al., 2020).

Nursing interventions and patient teaching

Proper observation and care after bone marrow study are essential. Patients with aplastic anemia are highly susceptible to infection; thus nursing interventions should be directed toward prevention. Adhere to strict aseptic techniques for dressing changes and intravenous (IV) site care. To prevent impaired skin and mucous membranes, avoid intramuscular injections, administration of rectal medications, and measurement of rectal temperatures. An air mattress can help protect the patient’s skin. In the presence of thrombocytopenia, observe carefully for any signs of bleeding and prevent any risk for injury. Monitor the patient’s urine and stool for occult or gross blood.

Patient problems and interventions for the patient with aplastic anemia include, but are not limited to, Inability to Tolerate Activity, related to inadequate tissue oxygenation; and Potential for Infection, related to increased susceptibility.

Patients with aplastic anemia need to know how to protect themselves from excessive bleeding. Help the patient maintain a balance between rest and activity. Discuss with the patient how to avoid infection, especially of the respiratory or urinary tract (see the Safety Alert box).

Safety Alert

Aplastic Anemia

• Avoid contact with those who have infection.

• Avoid enemas or other rectal insertions.

• Avoid excessive workload or heavy lifting, and ask for assistance with strenuous activity.

• Avoid intramuscular injections.

• Avoid picking or blowing the nose forcefully.

• Avoid sharing eating utensils and bath linens.

• Avoid trauma, falls, bumps, and cuts; avoid contact sports.

• Avoid use of aspirin or over-the-counter (OTC) aspirin preparations (anticoagulant effect).

• Bathe or shower every day (or every other day if skin is dry); keep perineal area clean.

• Decrease activity if shortness of breath, dizziness, or sensation of heaviness in extremities occurs.

• Eliminate intake of raw meats, fruits, or vegetables.

• Immediately report signs of infection to health care provider.

• Increase time necessary for routine care.

• Keep mouth clean and free of debris.

• Observe for signs such as blood in urine or stool and petechiae, and report these to health care provider.

• Prevent fatigue.

• Prevent hemorrhage.

• Prevent infection.

• Report signs of increased fatigue.

• Take frequent rest periods between activities of daily living (ADLs).

• Use a soft toothbrush or swab for mouth care.

• Use adequate lubrication and gentleness during sexual intercourse.

• Use an electric razor.

• Use good hand hygiene technique.

• Use good oral hygiene.

Prognosis

The prognosis for the patient with aplastic anemia depends on the cause. Aplastic anemia caused due to medications, toxins, and viruses is short term, and any complications can be treated. Aplastic anemia may or may not resolve in pregnant patients after delivery, and women may be susceptible to the disease during future pregnancies. Patients receiving bone marrow transplants fair better, but immunosuppressive therapies work well for patients unable to get a bone marrow transplant. Patients with severe chronic aplastic anemia that doesn’t respond to treatment has the poorest prognosis.

Iron Deficiency Anemia

Etiology and pathophysiology

Iron deficiency anemia is a condition that occurs when the mineral iron is not available to make healthy RBCs. Iron is a key ingredient in the construction of hemoglobin, which is a protein on the RBC and the carrier of oxygen. A decrease in the amount of hemoglobin in RBCs creates a concomitant decrease in oxygen saturation in blood, which then decreases the amount of oxygen available to all cells within the body. Common causes of iron deficiency anemia are excessive iron loss, a diet low in iron sources, bleeding from gastric or duodenal ulcers, esophageal varices, hiatal hernias, colonic diverticula, and tumors. The major sources of chronic blood loss are from the GI and genitourinary systems (Box 47.2).

Box 47.1 Signs of Hypovolemia

Box 47.2 Causes of Iron Deficiency Anemia

• Blood loss is a major cause of iron deficiency in adults. The major sources of chronic blood loss are from the gastrointestinal (GI) and genitourinary systems. Common causes of GI blood loss are peptic ulcers, gastritis, esophagitis, diverticulitis, hemorrhoids, and neoplasms. The average monthly menstrual blood loss is about 45 mL and causes the loss of 22 mg of iron.

• Daily iron intake from food and dietary supplements is adequate to meet the needs of men and older women, but it may be inadequate for those with higher iron needs (e.g., menstruating or pregnant women).

• Iron deficiency may develop from inadequate dietary intake, malabsorption, blood loss, or hemolysis (breakdown of red blood cells).

• Malabsorption of iron may occur after certain types of GI surgery and in malabsorption syndromes. Iron absorption occurs in the duodenum. Malabsorption of iron may involve disease of the duodenum, in which the absorption surface is altered or destroyed.

GI bleeding is often not apparent and may exist for a considerable time before being identified. Loss of 50 to 75 mL of blood from the upper GI tract is required for stools to appear black, or melenic. The color results from the iron in the RBCs. Blood losses related to menstruation or pregnancy are common causes of iron deficiency anemia in young women. Rarely, excessive losses occur through microhemorrhages into lung tissue or from intestinal parasites. Even without excessive blood loss, iron deficiency anemia can result when the body’s demand for iron exceeds its absorption, which commonly occurs in infants, young adolescents, and pregnant women. Iron deficiency anemia also may result from malabsorption of iron caused by diseases such as celiac disease and sprue. Subtotal gastrectomy may lead to iron deficiency caused by achlorhydria (loss of hydrochloric acid), occult bleeding, and decreased iron in post gastrectomy diets. Deficiency caused by poor dietary intake is rare in middle-aged adults.

Approximately 1 mg of every 10 to 20 mg (5% to 10%) of iron ingested is absorbed in the duodenum. For example, the average daily iron requirement for infants (older than 6 months), children, adolescents, and adults is between 7 mg and 11 mg. This amount of dietary iron meets the needs of men and older women, but it may be inadequate for people who have higher iron needs (e.g., ill children, pregnant and lactating women).

Clinical manifestations

Symptoms specific to iron deficiency anemia are pallor, glossitis (red, smooth tongue), and brittle nails. Patients may also crave non-nutritive substances such as ice, dirt, and starch, known as Pica. Other signs and symptoms for iron deficiency anemia are those common to all other types of anemias as discussed previously.

Assessment

Collection of subjective data includes complaints of fatigue, cold hands and feet, headache, dizziness or lightheadedness, and reports of craving non-nutritive substances. Patients may also report chest pain and palpitations.

Collection of objective data includes noting the signs, including pallor and tachycardia. Fingernails may be fragile and shaped like the head of a spoon with a central depression and raised borders. Mucous membranes of the mouth may be inflamed (stomatitis), and sore lesions at the corners of the mouth known as angular cheilosis may be present.

Diagnostic tests

The peripheral blood counts show that RBCs, Hgb levels, and Hct are decreased, which points to the presence of anemia. The mean corpuscular volume (MCV) that measures the size and volume of the RBC is low. A low ferritin level is diagnostic for iron deficiency anemia as ferritin stores iron.

Medical management

Administration of iron salts such as ferrous sulfate often is required. In 3 weeks the Hct level should rise 5% to 15%, and the Hgb level should rise by 2 to 5 g/dL. For the body to incorporate 100 mg of iron per day, 900 mg/day is administered. Iron is administered orally or by injection. For patients unable to swallow pills, liquid ferrous sulfate can be administered. Ascorbic acid (vitamin C) has been shown to enhance iron absorption. Food sources of iron include meat, fish, poultry, eggs, green leafy vegetables, whole grains, and dried beans (Box 47.3).

When the patient cannot tolerate oral preparations of iron, parenteral iron therapy is used. The Z-track method of giving iron dextran intramuscularly is preferable to prevent skin staining. Iron sucrose is an IV drug frequently used to treat iron deficiency anemia.

Nursing interventions and patient teaching

Because treatment is directed toward diagnosis and alleviation of the cause, the patient interview is important. Medication therapy for iron replacement is initiated as ordered. Plan for rest periods when fatigue is present. Education about nutritional needs relative to the condition may prevent this anemia (see Box 47.3).

Box 47.3 Food Sources of Nutrients Needed for Erythropoiesis

Iron

Folic Acid

• Asparagus, broccoli

• Enriched and fortified breads and cereals

• Fish

• Green, leafy vegetables

• Legumes

• Meat

• Organ meats: Liver

• Whole-grain breads and cereals

Vitamin B₁₂

• Eggs

• Milk and cheese

• Muscle meats

• Organ meats: liver and kidney

Amino Acids

• Eggs

• Fish

• Legumes

• Meat

• Milk and milk products (cheese, ice cream)

• Nuts

• Poultry

Vitamin C

• Cantaloupe

• Citrus fruits

• Leafy, green vegetables

• Strawberries

Explanation of the side effects of iron therapy is essential to alleviate distress and to extend the therapy for the necessary time (see the Health Promotion box). The patient must know which signs and symptoms are significant and must be reported to the health care provider. Diarrhea or nausea is significant, but black, tarry stools are not (these are expected with iron therapy).

Prognosis

The prognosis is usually good with correction of the underlying cause and compliance with the medical treatment.

Megaloblastic Anemia

Etiology and pathophysiology

Megaloblastic anemia, a type of macrocytic anemia, occurs due to the deficiency of two important vitamins, folic acid (also known as folate, folacin, and vitamin B9) and vitamin B₁₂ (cyanocobalamin). In the absence of either folic acid, vitamin B₁₂, or both, cells within the bone marrow undergo impaired DNA synthesis, which results in the production of cells that are too big (megaloblasts), not fully developed, and cells that do not live as long as normal RBCs. These megaloblasts crowd the bone marrow, resulting in self-death, as well as the crowding out of and destruction of WBCs and platelets. The cells that do manage to make it out of the bone marrow are fewer in numbers, thus resulting in the finding of pancytopenia (decreased levels of leukocytes,

Health Promotion

Iron Administration

• Check for constipation or diarrhea. Record color (iron turns stools green to black) and amount of stool.

• Dilute liquid iron preparations in juice or water, and administer with a straw to avoid staining teeth. Provide oral hygiene after taking.

• Do not administer with antacids, because they reduce the absorption of iron.

• Dosages are determined by the elemental iron content of the preparation.

• If a dose is missed, continue with the schedule; do not double a dose.

• If side effects develop, the dose and type of iron supplement may be adjusted. Some people cannot tolerate ferrous sulfate because of the effects of the sulfate base. Ferrous gluconate may be an acceptable substitute.

• Iron is absorbed best from the duodenum and proximal jejunum. Therefore enteric-coated or sustained-release capsules, which release iron farther down in the GI tract, are counterproductive; they are also more expensive.

• Iron is best absorbed in an acidic environment. To avoid binding the iron with food, iron should be taken about an hour before meals, when the duodenal mucosa is most acidic. Taking iron with vitamin C (ascorbic acid) or orange juice, which contains ascorbic acid, also enhances iron absorption. Gastric side effects, however, may necessitate ingesting iron with meals.

• Iron is toxic, and caution must be taken to store iron preparations out of a child’s reach.

• Iron may interfere with absorption of oral tetracycline antibiotics (quinolones). Do not take within 2 hours of each other.

• Iron preparations supplement the body’s natural iron stores.

• Iron supplements may be contraindicated in peptic ulcer disease.

• Side effects include gastrointestinal (GI) upset (nausea, vomiting), constipation or diarrhea, and green to black stools. Elixir may stain teeth.

thrombocytes, and erythrocytes) on CBC examination. The large RBCs that are released into circulation carry less hemoglobin, which means there is a concomitant decrease in the amount of oxygen carried.

Folic acid is present in foods such as green vegetables (broccoli, Brussel sprouts, kale, cabbage, and spinach), fruits (mango, kiwi, and pomegranate), and liver. Patients who rarely consume uncooked vegetables are at higher risk for folic acid deficiency. The recommended average intake for adults is 400 μg and pregnant women 600 μg. Approximately 5 mg of folic acid is stored in the liver. This store is depleted in 3 to 4 months’ time if diet is deficient of all folic acid. Besides an inadequate diet, folic acid deficiency can occur in the presence of alcoholism, malabsorption disorders (celiac disease, jejunal resection), malnutrition, due to medications such as anticonvulsants or anticancer agents, hemodialysis, and hemolysis (Hariz & Bhattacharya, 2020).

Vitamin B₁₂ is obtained through dietary sources such as meat, fish, eggs, and dairy products. Two to 3 mg of vitamin B₁₂ is stored in the liver and in the absence of dietary vitamin B₁₂, the amount of vitamin B₁₂ stored in the liver would deplete over the span of 2 to 4 years. Vitamin B₁₂ deficiency can develop in patients who are strict vegans who do not consume any dairy or meat products. Another cause of vitamin B₁₂ deficiency is malabsorption. Oral absorption of vitamin B₁₂ occurs with the help of intrinsic factor that is produced by the parietal cells in the gastric mucosa. Vitamin B₁₂ and intrinsic factor travel to the duodenum and jejunum where they are bound together. The bound intrinsic factor-vitamin B₁₂ complex is then absorbed by the terminal portion of the ileum. Diseases such as Crohn affect the ileum and would lead to a decrease in the absorption of vitamin B₁₂. Other disorders that affect vitamin B₁₂ absorption include bariatric surgery, gastrectomy, chronic use of histamine blockers, overuse of antacids or proton pump inhibitors, Zollinger-Ellison syndrome, and pancreatic insufficiency. Patients may also be deficient in vitamin B₁₂ due to a deficiency in the production of intrinsic factor. Without intrinsic factor, orally consumed vitamin B₁₂ is unable to be absorbed. Anemia that occurs due to a lack of intrinsic factor is known as pernicious anemia.

Clinical manifestations

The symptoms from megaloblastic anemia are insidious and may not manifest until the stores of folic acid, vitamin B₁₂, or both are depleted. Symptoms common to all types of anemia are also present in megaloblastic anemia, such as weakness, fatigue, lightheadedness, and palpitations. These symptoms may not manifest until later. Most of the symptoms of folic acid and vitamin B₁₂ deficiency are similar except that vitamin B₁₂ deficiency includes neurological symptoms.

Symptoms common to folic acid or vitamin B₁₂ deficiency include jaundice, angular cheilosis, brittle ridged concave nails, and sore red beefy tongue. Some patients may experience GI symptoms such as weight loss, loss of appetite, nausea, constipation, or diarrhea.

Neurological symptoms present in vitamin B₁₂ deficiency include confusion, problems concentrating, loss of balance, numbness and tingling in hands and feet, hallucinations, and psychosis.

Assessment

Subjective data include the patient’s complaints of palpitations, nausea, flatulence, constipation, diarrhea, and indigestion. The patient may complain that the tongue is tender and burning. Weakness and difficulty in swallowing (dysphagia) may occur. Neurologic symptoms include tingling of the hands and feet and loss of the sense of body position (impaired proprioception).

Collection of objective data includes observation of a smooth and erythematous tongue, brittle ridged concave nails, palpitations, and angular cheilosis. Cerebral signs include mental disorientation, personality changes, and behavior problems. Severe neurologic impairments can result, including partial or total paralysis from destruction of the nerve fibers of the spinal cord. If not treated promptly, the neurological symptoms of vitamin B₁₂ deficiency may become permanent.

Diagnostic tests

In addition to the patient’s signs and symptoms, several laboratory tests are used to diagnose megaloblastic anemia. The CBC is the most common test and helps in the preliminary diagnosis with the result of low RBC, Hgb, and Hct levels. An examination of the MCV will reveal a value greater than 100 fL (femtoliters). Further testing is required for an accurate diagnosis. These tests include reticulocyte count, which measures the number of RBCs in the blood, indicating if the bone marrow is producing RBCs; serum folate, serum megaloblastic anemia profile (discussed under laboratory and diagnostic tests section); intrinsic factor antibody test (its presence indicates pernicious anemia); and bone marrow aspiration and biopsy if other tests are not conclusive. Patients may also need further testing to determine the cause of the vitamin deficiency if it is not related to diet.

Medical management

Folic acid deficiency is treated with oral supplements of folic acid as well as a folate enriched diet. For patients with folic acid deficiency related to malabsorption, folic acid is administered intramuscularly and can also be administered intravenously. The recommended daily supplementation for folic acid is 1 mg per day. Patients who drink alcohol may need to ingest higher dosages of folic acid if they continue to keep drinking. It takes approximately 2 to 3 months to replace the depleted folic acid stores in the liver.

Vitamin B₁₂ deficiency is treated with oral supplementation and changes in diet regimen. Strict vegans need to consume foods such as almond milk, soy milk, plant-based meats, and fortified cereals as sources of vitamin B₁₂. If the presence of vitamin B₁₂ deficiency is due to lack of intrinsic factor or malabsorption issues, the patient is given intramuscular vitamin B₁₂ injections on a monthly basis. A resolution of malabsorption derived vitamin B₁₂ deficiency may allow the patient to ingest oral vitamin B₁₂ after the cause of malabsorption is identified and treated. However, vitamin B₁₂ deficiency arising from a lack of intrinsic factor will require intramuscular vitamin B₁₂ injections for life.

Nursing interventions and patient teaching

The nursing interventions depend to some extent on the stage of the disease. A symptomatic approach is appropriate. When the patient is confined to the hospital, the nurse checks vital signs every 4 hours and performs special mouth care several times daily. The diet should be high in protein, vitamins, and minerals. Anemic patients are especially sensitive to cold, so additional lightweight, warm blankets may be needed. Interventions should conserve energy and prevent injury. The room temperature may have to be increased for the patient’s comfort.

Patient problems and interventions for the patient with megaloblastic anemia include but are not limited to the following:

Patient Problem	Nursing Interventions
Insufficient Nutrition, related to: • Sore mouth and tongue • Diarrhea • Constipation	Administer folic acid prescribed to promote the production of erythrocytes Administer vitamin B₁₂ and other medications prescribed to promote production of erythrocytes Instruct patient on balanced diet high in protein, vitamins, and iron, such as red meat, dairy products, and eggs, to increase intake of vitamin B₁₂ Instruct patient to eat uncooked vegetables and liver to increase folate levels Provide meticulous and frequent oral hygiene to promote improved appetite and prevent infection Provide six to eight small meals daily to conserve energy and decrease gastrointestinal distress Monitor patient’s bowel movements, noting color, consistency, and amount

Patient Problem

Nursing Interventions

Insufficient Nutrition, related to:

• Sore mouth and tongue

• Diarrhea

• Constipation

Administer folic acid prescribed to promote the production of erythrocytes

Administer vitamin B₁₂ and other medications prescribed to promote production of erythrocytes

Instruct patient on balanced diet high in protein, vitamins, and iron, such as red meat, dairy products, and eggs, to increase intake of vitamin B₁₂

Instruct patient to eat uncooked vegetables and liver to increase folate levels

Provide meticulous and frequent oral hygiene to promote improved appetite and prevent infection

Provide six to eight small meals daily to conserve energy and decrease gastrointestinal distress

Monitor patient’s bowel movements, noting color, consistency, and amount

Emphasize the importance of continued supplementation of the missing vitamin and adequate diet intake containing the vitamin. Help patients understand that pernicious anemia will require the patient to take monthly vitamin B₁₂ injections for life. Patients with neurological deficits due to vitamin B₁₂ deficiency may need help with ADLs, frequent rest periods, and are taught to avoid excessive heat or cold.

Prognosis

This condition, if untreated, can be considered terminal in 1 to 3 years. With treatment, folic acid deficiency results in cessation of symptoms. Patients with vitamin B₁₂ deficiency who have been left untreated for a while may never fully recover from the neurological manifestations.

Hemolytic anemias

Hemolytic anemias occur when erythrocytes are destroyed faster than they can be produced. Sickle cell anemia is a type of hemolytic anemia.

Sickle Cell

Sickle cell trait

Sickle cell trait is a term given to an individual that has a genetic mutation for hemoglobin that gives rise to the production of an abnormal hemoglobin. The normally occurring hemoglobin is referred to as HbA, and it remains soluble in water and does not precipitate in the presence of hypoxia. This aspect allows RBCs to remain in solution and maintain their biconcave shape. The hemoglobin caused by the gene mutation is referred to as HbS, and in the presence of hypoxia, all the hemoglobin S contained in a RBCs starts to polymerize or link up, causing the RBC to become sickle shaped. This sickle-shaped RBC then falls out of solution and starts to clump with other RBCs (Mangla, Ehsan, & Maruvada, 2020). Patients with sickle cell trait inherit the normal gene for hemoglobin from one parent and an abnormal hemoglobin gene from the other parent. Therefore, genetically speaking, a patient with sickle cell trait is heterozygous for hemoglobin. The presence of a normal hemoglobin (HbA) gene offsets many of the effects of the abnormal hemoglobin gene, therefore these patients do not have sickle cell disease and are not susceptible to vaso-occlusive crisis. However, some sickle cell trait patients when exposed to favorable conditions such as severe hypoxia, dehydration, hypothermia, hyperthermia, and release of inflammatory cells experience sickling of RBCs. The sickled RBCs clog tiny capillaries most especially in the bones and attract inflammatory cells and platelets that cause RBCs to adhere even more. Any organ may be affected, and multiple attacks could cause damage due to ischemia. The occurrence of sickle cell trait is 9% among the African American population and 0.2% among Caucasians. It is estimated that there are approximately 300 million people worldwide with sickle cell trait, and migration patterns will cause the number of people with sickle cell trait in the Western part of the world to rise. In all 50 US states, babies are tested for sickle cell trait before discharge (Ashorobi, Ramsey, Yarrarapu, et al., 2020).

Sickle Cell Anemia

Etiology and pathophysiology

Sickle cell disease is the most common genetic disorder in the United States, predominantly affecting those of African or Eastern Mediterranean heritage. It is estimated that sickle cell disease affects approximately 100,000 Americans or 1 out of every 365 African Americans (Mangla, Ehsan, & Maruvada, 2020). Sickle cell disease occurs when an individual inherits an abnormal hemoglobin S gene from both parents. In the presence of hypoxia, hemoglobin S polymerizes to form a sickle shape that then causes the RBC to fall out of solution and clump with other sickled RBCs. As the disease is inherited, patients with sickle cell disease manifest symptoms after 6 months of age, as the presence of remaining fetal hemoglobin provides protection against sickling (Mangla, Ehsan, & Maruvada, 2020). The most common symptom experienced is a vaso-occlusive crisis, which occurs when sickled RBCs clump together and get trapped in microcirculation along with trapping other cells such as leukocytes. The entrapment causes tissue hypoxia, inflammation, and necrosis to the tissue or organ being perfused. When circulation is restored, the clumped cells have lysed, which releases substances that cause further damage to the vessels in the area. The patient experiences severe pain and symptoms of the tissue or organ experiencing ischemia. Patients may also experience sequestration crisis, which occurs when sickled cells pool in an organ. The most common site for this occurrence is the spleen, and many children experience splenic infarction. In adults, the organs most likely involved in sequestration include the liver and the lungs. In addition, the hemolysis of RBCs causes a decrease in RBCs, Hgb, and HCT levels, and the patient experiences the signs and symptoms of anemia. Sickling of RBCs occurs during times of physical and emotional stress. As the sickling process takes time, quick administration of oxygen reverses the sickling process thus decreasing or arresting crisis.

Clinical manifestations

Symptoms of sickle cell anemia vary over time and can be multifactorial. Most patients with sickle cell anemia suffer from chronic anemia due to the hemolysis of sickled cells, which have a lifespan of 10 to 20 days. The chronic anemia leads to decreased oxygen levels and constant fatigue. Patients experience pain crisis brought on by ischemia secondary to sickled cells. The pain varies and can last from a few hours to weeks. Severe pain may require hospitalization. Infections and the signs and symptoms of infection are common due to a damaged spleen unable to perform its immune functions. Sickled cells may also damage the retina, leading to vision problems (Mayo Clinic, 2020).

Assessment

Collection of subjective data begins with assessing the patient’s knowledge and feelings about the disease and factors that appear to precipitate crisis or exacerbate signs and symptoms. Fatigue may be reported when anemia is severe. The primary symptom associated with sickling is pain in the joints, especially those of the hands and feet. Abdominal pain is common with swelling of the spleen and engorgement of vital organs. Hypoxia occurs as fever and pain increase, causing the patient to breathe rapidly. A male patient may have a continuous painful erection (priapism) from impaired blood flow out of the erect penis. The pain associated with these attacks often is described as deep, gnawing, and throbbing.

Further collection of objective data includes observing for abdominal enlargement and jaundice, edema of the extremities, and signs of hemorrhage. As a result of the accelerated RBC breakdown, the patient has a characteristic clinical finding of hemolysis (jaundice, elevated serum bilirubin levels).

Diagnostic tests

Electrophoresis of Hgb from a patient with sickle cell anemia is specific for detecting sickle cell crisis or anemia. More than 80% of sickle cell Hgb as shown by electrophoresis is Hb-S, not Hb-A. A stained blood smear detects anemia only. The RBC, Hct, and Hgb levels are below normal values. WBCs are increased with infection. Skeletal x-rays demonstrate bone and joint deformities and flattening. MRI may be used to diagnose a stroke caused by occluded cerebral vessels from sickled cells.

Medical management

Sickle cell anemia has no specific treatment. Treatment is symptomatic, alleviating the symptoms that result from complications. Treatment should include adequate hydration, antibiotics for infections, and nonsteroidal anti-inflammatory (NSAIDs) medications for pain. Haemophilus influenzae, pneumococcal conjugate, meningococcal, and hepatitis immunizations should be administered to prevent infections. A bone marrow transplant, which infuses healthy stem cells into the bone marrow, may be performed in select patients to treat severe cases of the disease. The challenge is finding a match for transplantation (Mayo Clinic, 2020). Sickle cell crisis may require hospitalization. Oxygen may be administered to alter hypoxia and control sickling. Encourage rest and administer fluids and electrolytes intravenously to reduce blood viscosity and maintain renal function. Use analgesics to treat pain. Sickle cell crisis pain often is undertreated. The nurse needs a clear understanding of the disease process and of current approaches to pain management.

Opioid pain management is often necessary when NSAIDs are no longer effective. Patient-controlled analgesia may be used during an acute crisis. After discharge, patients may continue taking oral opioid analgesics for a period of time. Blood transfusion of packed RBCs may be necessary to treat severe anemia. However, frequent transfusions can cause high levels of iron to build up in the body. Chelation therapy, a medicine to reduce the amount of iron in the body and the problems that iron overload causes, may be prescribed (NHLBI, 2017). These patients have an increased need for folic acid, so it is important that they take daily supplements. Iron therapy generally is not suggested.

Another medication, hydroxyurea, currently is being prescribed as maintenance therapy to help prevent anemia and to prevent acute episodes of pain (Mayo Clinic, 2020). This medication boosts the levels of fetal Hgb (Hb-F). This lowers the concentration of Hb-S within a cell, resulting in less polymerization of the abnormal Hgb.

Nursing interventions and patient teaching

Supportive treatment depends on the signs and symptoms: hydration and analgesia during crises, and dilution of blood with increased fluid intake to reverse sickling. Monitoring the transfusion therapy for evidence of transfusion reaction is vital. Attention to fever and infection is important. Genetic counseling is indicated.

A patient problem and interventions for the patient with sickle cell anemia include but are not limited to the following:

Patient Problem	Nursing Interventions
Recent Onset of Pain, related to thrombotic crisis	Place patient in proper anatomic alignment, and protect joints Position patient by slow, gentle handling Apply warmth with soaks or compresses to relieve discomfort Give analgesics on a fixed time schedule to maintain a steady serum drug level, which improves pain control, minimizes complications, and decreases anxiety Medications may be administered by the nurse or a patient-controlled analgesic infusion pump provides a constant, low-dose infusion of an opioid for excellent pain control

Patient Problem

Nursing Interventions

Recent Onset of Pain, related to thrombotic crisis

Place patient in proper anatomic alignment, and protect joints

Position patient by slow, gentle handling

Apply warmth with soaks or compresses to relieve discomfort

Give analgesics on a fixed time schedule to maintain a steady serum drug level, which improves pain control, minimizes complications, and decreases anxiety

Medications may be administered by the nurse or a patient-controlled analgesic infusion pump provides a constant, low-dose infusion of an opioid for excellent pain control

Alert the patient to the need for family testing to determine the presence of Hb-S; genetic counseling is available for carriers. Explain how to avoid sickle cell crises: avoid high altitudes, flying in unpressurized planes, dehydration, extreme temperatures, iced liquids, alcohol, and vigorous exercise; use stress reduction methods. Patients should not smoke and should protect extremities from injury because of impaired circulation. Patients with sickle cell disease have frequent problems with infections. It is important for the patient to remain current with vaccinations and take prophylactic antibiotics to protect against these infections. Explain that young pregnant women with sickle cell anemia have a high risk for developing pulmonary and/or renal complications. Alert the patient to the signs and symptoms of increased intracranial pressure and to the need to blow the nose gently, avoid coughing, and avoid straining on elimination.

Practice ROM exercises with the patient and encourage regular physical activity to prevent bone demineralization. Instruct the patient in the need for a balance between rest (physical and mental) and activity, such as ROM and isometric exercises. Also discuss the principles of good nutrition, such as the importance of protein, calcium, vitamins, and adequate fluids. Provide patient education on how to monitor oral intake, urinary output, and urine protein.

Prognosis

The prognosis for sickle cell disease is guarded with treatment aimed at ensuring a good quality of life. Vaso-occlusive crisis, sequestration, and chronic anemia cause pain disability, depression, economic loss, infections, and joint damage. Patients are disabled for life, and premature death is common (Mangla, Ehsan, & Maruvada, 2020).

Hypovolemic anemia (anemia resulting from blood loss)

Etiology and pathophysiology

Secondary anemia occurs when deficiencies in RBCs and other components are caused by an abnormally low circulating blood volume resulting from acute or chronic blood loss. Loss of blood decreases the amount of circulating fluid and Hgb and thus decreases the amount of oxygen carried to the body tissues. The tissues must have oxygen to survive. The average adult has an approximate total blood volume of 6000 mL (6 L [12 pints]) and can tolerate a loss of up to 500 mL. Blood loss of 1000 mL or more in an adult can have severe consequences. This level of reduction in total blood volume can lead to hypovolemic shock. Such a loss usually is related to internal or external hemorrhage caused by a surgical procedure, GI bleeding, menorrhagia (abnormally prolonged menstrual bleeding), trauma, or severe burns. The rapidity of blood loss is related to the severity and number of signs and symptoms.

Clinical manifestations

Signs and symptoms include restlessness; a subtle rise in respiratory rate; weakness; stupor; irritability; a pale, cool, moist skin; and a rapid, thready pulse (see Box 47.1). Excessive blood loss results in shock. Shock occurs when there is a deprivation of oxygen and nutrients to organs. Hemorrhagic blood loss results in a decrease in blood volume. In shock, vasoconstriction occurs in blood vessels to noncritical organs such as skin, muscles, and intestines. This decreases the blood flow to these organs and shunts blood to the vital organs such as the heart and the brain.

The greater the amount of blood loss, the more the heart rate and blood pressure are affected. The body is able to compensate for blood loss less than 15% or 750 mL. The initial symptom may be only tachycardia, although there can be an initial rise in systolic blood pressure, then a fall to below 80 mm Hg. At 20% to 25% blood loss, tachycardia and mild to moderate hypotension are present. With a loss of 40% or greater (2000 mL), all clinical signs and symptoms of shock are present.

Hypovolemic anemia in a child often results in differing signs and symptoms initially than in adults. The child may be alert and have normal blood pressure, pulse, and perfusion initially. Signs and symptoms may progress to cool and clammy skin, hypotension, tachycardia, tachypnea, and reduced urine output. Treatment is similar to adults. IV fluid administration is the first treatment. If fluid administration is not effective, blood administration may be necessary.

The patient’s clinical signs and symptoms of hypovolemic anemia are equally as important as the laboratory values. Be alert to the patient’s expression of pain. Internal hemorrhage may cause pain because of tissue distention, organ displacement, and nerve compression. Pain may be localized or referred (referred pain is felt at a place other than where the damage actually is). Decreased RBC, Hgb, and Hct levels decrease for 24 to 48 hours after blood loss until the plasma volume is replaced with blood or blood components (Maakaron, 2019). The severity of the patient’s signs and symptoms correlates with the severity of the blood loss.

Assessment

Subjective data commonly include complaints of thirst, weakness, irritability, and restlessness.

Objective data include decreased blood pressure; rapid, weak, thready pulse; and rapid respirations. Cold, clammy skin with pallor is noted. Oliguria (urine output less than 400 mL in 24 hours) is often evident. Mental disorientation and physical collapse with prostration can occur.

Diagnostic tests

When blood loss is sudden and plasma volume has not yet had a chance to increase, the loss of RBCs is not reflected in laboratory data, and values may seem normal or high for 2 to 3 days. However, once the plasma is replaced, the RBC mass is less concentrated. RBC, Hgb, and Hct levels are severely decreased, often to half the normal values.

Medical management

In the case of massive hemorrhage, measures are taken to stop the blood loss and treat for shock and lost volume. Severe hemorrhaging often results in the need for mechanical ventilation. Oxygen therapy restores oxygen that is less available because of decreased Hgb in the blood. To replace fluid volume, IV saline is administered. In severe fluid volume depletion, a bolus of 2 L of normal saline (i.e., a 0.9% NaCl solution) is given. Current guidelines from the American Association of Blood Banks (AABB) recommend transfusing packed RBCs when the Hgb falls below 7 g/dL for the patient in an acute care setting or in the intensive care setting (ICU), and when the Hgb falls below 8 g/dL for the patient who is symptomatic for anemia, for the preoperative and postoperative cardiac and orthopedic patient, and the patient with cardiovascular disease. Often platelets, fresh frozen plasma (FFP), or cryoprecipitate is included in the treatment to control hemorrhage (Maerz, 2019).

Monitor the Hgb level to note the effectiveness of the treatment. One unit of packed RBCs should increase the Hgb level by 1 g/dL or raise the Hct by 3%. The patient also may need supplemental iron because the availability of iron affects the marrow production of erythrocytes. Oral or parenteral iron preparations often are administered.

Nursing interventions and patient teaching

Monitor blood and fluid restoration and identify blood loss sites to control the bleeding. Keep the patient flat and warm. Take vital signs at frequent intervals (every 15 minutes or more frequently) until the systolic blood pressure is above 90 mm Hg. Take precautions to prevent injury to a restless or disoriented patient. Measure intake and output (I&O), with careful monitoring of urinary output for oliguria caused by decreased renal perfusion. The decrease in urinary output correlates to the amount of blood lost.

If hemorrhage is caused by a chronic problem, teach the patient to monitor bleeding amounts and associated factors and to report to the health care provider immediately for treatment.

Prognosis

Without treatment, death results. With aggressive treatment, the prognosis is favorable.

Polycythemia (Erythrocytosis)

Etiology and pathophysiology

The two types of polycythemia are primary polycythemia (polycythemia vera) and secondary polycythemia. Their causes and pathophysiology differ, although their complications and clinical manifestations are similar and yet at times different.

Polycythemia vera is a rare blood disorder that occurs due to an acquired, not inherited, mutation in the JAK2 gene (Accurso, Santoro, & Mancuso, 2020) that affects a precursor blood forming cell in the bone marrow. The mutation results in an overproduction of particularly RBCs but also WBCs and platelets because a precursor cell in the myeloid line is affected. The cause of the genetic mutation is unknown, and even though there is no line of direct inheritance, oftentimes more than one family member is afflicted with the disease. Polycythemia vera occurs more commonly in men aged 60 years and older and has a slow progression and symptom onset. An overproduction of cells from the bone marrow results in increased blood viscosity and volume.

Secondary polycythemia can occur at any age and is caused by chronic hypoxia. Chronic hypoxemia occurs in patients for multiple reasons including living in high altitudes where atmospheric oxygen is lower, pulmonary disease, cardiovascular disease, and patients with obstructive sleep apnea. Chronic hypoxemic blood filters through the kidney, which senses low oxygen saturation. In response, the kidney releases erythropoietin, which stimulates the bone marrow to produce more RBCs. Secondary polycythemia occurs due to a physiological response where the body compensates for lower oxygen levels by producing more erythrocytes; no pathological process is in evidence.

Clinical manifestations

Polycythemia vera leads to an increased number of erythrocytes within the bloodstream. These extra RBCs cause the blood to be thicker or have an increased viscosity. Blood that is more viscous tends to be more sluggish, which affects multiple organs within the body. A sluggish circulatory process results in hypercoagulability. High platelet counts also contribute to the formation of thrombi. Patients are at an increased risk for blood clots in the deep veins of the arms and legs (deep vein thrombosis [DVT]), the lungs (pulmonary embolism [PE]), the heart (MI), and the brain (cerebrovascular accident [CVA]) (NIH, 2017a). In addition, the viscosity or stickiness of blood results in congestion of tissues all over the body. Viscous blood is harder to pump and therefore results in elevated blood pressure and left ventricular hypertrophy. Angina or congestive heart failure may also result due to thicker blood and tendency for platelets to clump in coronary vessels. The increase in RBCs causes blood to become a hypertonic solution whereby fluid is pulled from cells into the arteries and veins resulting in chronic hypervolemia. Hepatomegaly and splenomegaly, the result of organ engorgement, may contribute to patient complaints of satiety and fullness.

Secondary polycythemia results in an excess of erythrocytes only. Like polycythemia vera, patients are at risk for blood clots, left ventricular hypertrophy, congestive heart failure, and chronic hypervolemia. Hepatomegaly and splenomegaly are also common.

Assessment

Subjective symptoms may include largely asymptomatic patients to those presenting with a host of symptoms. Patient with either polycythemia vera or secondary polycythemia may complain of fatigue, abdominal fullness or discomfort, headaches, ringing in the ears, blurred vision, burning or pins-and-needles sensations in the feet, and difficulty breathing. Patients with polycythemia vera may complain of itchy skin or pruritus due to the high basophil count, gout, and bleeding or bruising.

Objective data for patients with polycythemia vera includes high blood pressure, shortness of breath, signs of clots, and an erythemic appearance to the skin. An ultrasound of the abdomen may reveal hepatomegaly and/or splenomegaly.

Diagnostic tests

CBC for patients with polycythemia vera reveals high levels of RBCs, Hgb, HCT, WBCs, and thrombocytes. Bone marrow examination reveals hypercellularity in WBCs, RBCs, and platelets. A genetic test reveals a mutation in the JAK2 gene for patients with polycythemia vera. However, erythropoietin levels in plasma are low in patients with polycythemia vera but high in patients with secondary polycythemia or erythrocytosis. The high erythropoietin level in secondary polycythemia is a distinguishing factor. The RBC, Hgb, and HCT levels will also be high in secondary polycythemia.

Medical management

In both polycythemia vera and secondary polycythemia, the mainstay of therapy includes decreasing blood viscosity. In secondary polycythemia, this is accomplished by prescribing low-dose aspirin and repeated phlebotomy. In phlebotomy, roughly 500 mL of blood is removed at a time until the hematocrit level in the body is maintained between 45% and 48%. Phlebotomy immediately relieves certain symptoms such as headaches, ringing of the ears, and dizziness. Patients with polycythemia vera may be managed with phlebotomy alone for many years.

Nursing interventions and patient teaching

Patients with secondary polycythemia are educated to control and avoid situations that lead to low oxygen levels. Therefore, patients are educated on smoking cessation, controlling chronic pulmonary disease, and being compliant with treatment for cardiovascular diseases. In addition, it is important for patients with secondary polycythemia and polycythemia vera to be compliant with taking aspirin as prescribed and attending all appointments for phlebotomy treatments.

The nurse monitors patients with polycythemia for signs and symptoms of fluid overload and dehydration. Intake and output are strictly monitored. A dietitian may assess the patient for nutritional deficiencies due to abdominal discomfort and feelings of fullness. Patients are encouraged to conduct passive or active leg exercises and ambulate as much as tolerated. Patients taking myelosuppressive drugs need to be monitored regularly for side effects. Laboratory studies such as CBC are also conducted regularly. The nurse needs to educate patients regarding symptoms of potential complications such as those associated with DVTs, strokes, PE, and MI.

Patient problems and interventions for the patient with polycythemia vera include but are not limited to the following:

Patient Problem	Nursing Interventions
Compromised Blood Flow to Tissue (cardiopulmonary, cerebral, gastrointestinal, and peripheral), related to: • Hyperviscosity of fluid • Potential bleeding	Keep patient in a comfortable position, turning frequently to relieve pressure Elevate head of bed, keeping legs in a nondependent position Use range-of-motion exercises to stimulate circulation Assess peripheral pulses and color and temperature of extremities every 4–6 h Assess for blood in urine and stools Assess for thrombus formation Monitor laboratory studies If patient has a bleeding tendency, avoid invasive procedures when possible Avoid trauma; provide education related to activities of daily living Monitor blood pressure Monitor for signs of dehydration such as skin turgor, dry mucus membranes

Patient Problem

Nursing Interventions

Compromised Blood Flow to Tissue (cardiopulmonary, cerebral, gastrointestinal, and peripheral), related to:

• Hyperviscosity of fluid

• Potential bleeding

Keep patient in a comfortable position, turning frequently to relieve pressure

Elevate head of bed, keeping legs in a nondependent position

Use range-of-motion exercises to stimulate circulation

Assess peripheral pulses and color and temperature of extremities every 4–6 h

Assess for blood in urine and stools

Assess for thrombus formation

Monitor laboratory studies

If patient has a bleeding tendency, avoid invasive procedures when possible

Avoid trauma; provide education related to activities of daily living

Monitor blood pressure

Monitor for signs of dehydration such as skin turgor, dry mucus membranes

Prognosis

Polycythemia vera is a chronic, life-shortening disorder. Although the incidence is small, leukemia and lymphomas develop in some patients with polycythemia vera. This may occur as a result of the chemotherapeutic drugs used to treat the disease or may be secondary to a disorder in the stem cells that progresses to leukemia. The major cause of morbidity and mortality from polycythemia vera is thrombosis.

Disorders Associated With Leukocytes

Agranulocytosis

Etiology and pathophysiology

Agranulocytosis is a potentially life threatening and fatal condition that occurs when there is a severe reduction in the number of granulocytes, particularly neutrophils, circulating in blood. The absolute neutrophil count (ANC) is normally 3000 to 7000 cells/mm³. In agranulocytosis, the neutrophil count may be as low as 100 cells/mm³. This decrease in neutrophils is also represented in the WBC count, as 60% to 70% of WBCs are neutrophils. Therefore, a reduction in neutrophils causes a concomitant reduction in leukocytes. The patients’ CBC results indicate neutropenia as well as leukopenia.

Agranulocytosis can be inherited or acquired. The inherited form manifests in infancy due to genetic abnormality and causes infantile genetic agranulocytosis. The acquired form of agranulocytosis is rare and occurs in 6 to 8 per million population per year. It also occurs more commonly in women than in men. Of these cases, 70% of agranulocytosis occurs due to medications. Common medications implicated in the cause of agranulocytosis include naproxen, propylthiouracil, procainamide, captopril, clozapine, amitrypiline, chloroquine, phenytoin, penicillin, cephalosporin, cimetidine, ranitidine, chemotherapeutics, and zidovudine, to name a few (Sedhai, Lamichhane, & Gupta, 2020). These medications cause bone marrow suppression, which reduces production of immune cells. Viral and bacterial infections have also been implicated as a cause of agranulocytosis. Other disease processes such as aplastic anemia, leukemia, and myelodysplastic syndrome may also result in this disease.

Clinical manifestations

Fever, chills, headache, and fatigue are symptoms associated with infection and the inflammatory process. Patients present with sores in their mouth, throat, or gums and recurrent chronic infections of the throat and skin. Bronchial pneumonia and urinary tract infections are complications that occur in the later stages.

Assessment

Subjective data include common complaints of fever, extreme fatigue, and infections that last for long periods of time. All medications taken, whether prescription or over-the-counter, are considered as possible causes of the condition. A thorough history may provide relevant cues to the cause of agranulocytosis.

Objective data include fever over 100.6°F (38.1°C). Erythema and pain from ulcerations may occur. Ulcerations are cultured for microorganisms. Lung and bronchial auscultation reveals crackles and rhonchi (course rattling sounds) because of trapped exudates.

Diagnostic tests

The initial workup requires a CBC with differential count that shows an ANC of 100 cells/m³ or less. A peripheral blood smear will reveal a marked decrease or absence of neutrophils. An examination of bone marrow will reveal a presence of large numbers of promyelocytes that, if matured, would have become granulocytes. For patients exhibiting infections, cultures can be obtained to determine the causative agent.

Medical management

The main objective of treatment is to alleviate the factors responsible for bone marrow depression and to prevent or treat infection. Medications contributing to the disease are immediately stopped. It takes roughly 1 to 3 weeks for medication-induced agranulocytosis to resolve (Sedhai, Lamichhane, & Gupta, 2020). Pathogens causing infections are promptly treated. Hematopoietic growth factors are used to stimulate production of granulocytes from the bone marrow. Granulocyte colony-stimulating factor (G-CSF) (filgrastim), pegfilgrastim, and granulocyte-macrophage colony-stimulating factor (GM-CSF) (sargramostim) are given subcutaneously or intravenously. Precautions to prevent infection in the neutropenic (immunocompromised) patient also may be instituted.

Nursing interventions and patient teaching

A patient with a compromised WBC system is highly susceptible to life-threatening infections. Nursing interventions are directed toward protecting the patient from potential sources of infection. Monitor the patient conscientiously to detect the earliest signs of infection so that therapy may be initiated promptly. Restrict visitors and prevent personnel with colds from caring for the patient. Meticulous hand hygiene and universal precautions by medical and nursing personnel and strict asepsis are mandatory.

A patient problem and interventions for the patient with agranulocytosis include but are not limited to the following:

Patient Problem	Nursing Interventions
Potential for Infection, related to depressed white blood cell (leukocyte) production	Institute neutropenic precautions Restrict visitors or medical personnel with bacterial or viral infections Provide instruction on handwashing to patient and visitors Monitor for signs and symptoms of infection Maintain standard precautions Use strict asepsis for procedures Avoid fresh flowers and plants Provide high-protein, high-vitamin, high-calorie soft diet to maintain nutritional status Avoid raw foods, such as sushi, Caesar salad dressing (may have raw eggs), blue cheese, and fruits that cannot be peeled or vegetables that cannot be well cleaned Encourage increased fluid intake to prevent dehydration Monitor vital signs to assess for signs of infection Observe the patient for extreme fatigue, sore throat or mouth, and fever as signs of infection Monitor laboratory values Relieve fever with tepid bath or cooling blanket Administer antibiotics as prescribed Provide hygiene with adequate rest periods

Patient Problem

Nursing Interventions

Potential for Infection, related to depressed white blood cell (leukocyte) production

Institute neutropenic precautions

Restrict visitors or medical personnel with bacterial or viral infections

Provide instruction on handwashing to patient and visitors

Monitor for signs and symptoms of infection

Maintain standard precautions

Use strict asepsis for procedures

Avoid fresh flowers and plants

Provide high-protein, high-vitamin, high-calorie soft diet to maintain nutritional status

Avoid raw foods, such as sushi, Caesar salad dressing (may have raw eggs), blue cheese, and fruits that cannot be peeled or vegetables that cannot be well cleaned

Encourage increased fluid intake to prevent dehydration

Monitor vital signs to assess for signs of infection

Observe the patient for extreme fatigue, sore throat or mouth, and fever as signs of infection

Monitor laboratory values

Relieve fever with tepid bath or cooling blanket

Administer antibiotics as prescribed

Provide hygiene with adequate rest periods

In patient teaching, discuss the use of frequent and meticulous oral hygiene to treat or prevent mouth and pharyngeal infection. Explain the need to avoid crowds, people with infectious diseases, and cold or hot environments; also teach signs and symptoms of infection and appropriate interventions. Explain the need for a soft, bland diet (if mouth ulcers are present) high in protein, vitamins, and calories. Encourage a balance between rest and activity to prevent fatigue and generalized weakness. Teach the patient to consult a medical provider before taking any over-the-counter medications.

Prognosis

Patients older than 65 years of age, ANC count of less than 100 μL, and pre-existing comorbidities such as renal, cardiac, respiratory, and systemic inflammatory diseases have poorer outcomes and a grim prognosis. Complications including worsening infections and sepsis contribute to adverse prognosis as well.

Leukemia

Etiology and pathophysiology

Leukemia is a malignant disorder of the hematopoietic system in which excessive numbers of abnormal leukocytes accumulate in the bone marrow and lymph nodes. Risk factors include genetics, viral infection, previous treatment with or exposure to radiation, chemotherapeutic agents, smoking, family history, and exposure to certain chemicals such as benzene (Mayo Clinic, 2018).

In leukemia, normal WBCs in the bone marrow are replaced with abnormal numbers and forms of rapidly dividing cells, which then spread to the circulation and infiltrate the lymph nodes, spleen, and other organs, including those of the central nervous system. Leukemic infiltration leads to problems such as hepatomegaly, splenomegaly, lymphadenopathy, bone pain, meningeal irritation, and oral lesions. Hematopoietic function is disrupted by incompetent bone marrow. Increased susceptibility to infection results.

Classification

Leukemia comes in multiple forms and is classified by identifying whether it is acute or chronic and what type of blood cell is involved. There are four common types of leukemia (ACS, 2018):

• Acute lymphocytic (lymphoblastic) leukemia (ALL): ALL affects lymphoid cells and grows quickly. Nearly 6000 new cases will be diagnosed annually. The majority of cases are in children under the age of 5 years and in males. After children, the next age group impacted are those over the age of 50 years.

• Acute myeloid leukemia (AML): AML affects myeloid cells and grows quickly. More than 19,000 new cases will be diagnosed annually. While it occurs in both adults and children, the majority of cases will be in adults.

• Chronic lymphocytic leukemia (CLL): CLL affects lymphoid cells (cells that become lymphocytes, often B cells) and usually grows slowly. Approximately 21,000 Americans will be diagnosed each year. The incidence is rare in children and young adults. Diagnosis is most commonly made in the seventh decade of life.

• Chronic myeloid leukemia (CML): CML affects myeloid cells (cells that become any type of blood cell other than lymphocytes) and usually grows slowly at first. Approximately 8500 cases are diagnosed each year. Most cases involve older adults.

Clinical manifestations

The clinical manifestations of leukemia vary. They relate to problems caused by bone marrow failure and the formation of leukemic infiltrates. Bone marrow failure results from (1) bone marrow overcrowding by abnormal cells and (2) inadequate production of normal marrow elements. The patient is predisposed to anemia and thrombocytopenia.

As leukemia progresses, fewer normal blood cells are produced. The abnormal WBCs continue to accumulate. The leukemic cells infiltrate the patient’s organs, leading to problems such as splenomegaly, hepatomegaly, lymphadenopathy, bone pain, meningeal irritation, and oral lesions. Enlarged lymph nodes and painless splenomegaly may be the first signs of the disease in some patients.

Diagnostic tests

A CBC is a test to check the number of WBCs, RBCs, and platelets. Leukemia also may cause low levels of platelets and Hgb, which is found inside RBCs. It can cause the WBC count to be low, elevated, or excessively elevated. Anemia and thrombocytopenia are noted. Bone marrow biopsy shows immature leukocytes. Chest radiographic examination may show mediastinal lymph node and lung involvement and bone changes. Lymph node biopsy reveals excessive blasts (immature cells). Peripheral blood evaluation and bone marrow examination are the primary methods of diagnosing and classifying the type of leukemia. Further studies such as lumbar puncture and CT scan can be performed to determine the presence of leukemic cells outside of the blood and bone marrow.

Assessment

Subjective data include patient history and physical assessment. Patients often have pain in bones or joints, fatigue, malaise, decreased activity tolerance, and irritability.

Objective data include those signs listed in clinical manifestations. Data include laboratory studies of WBCs with differential. Cultures of throat, urine, stool, and blood are obtained to determine which organisms are present. Abnormalities of skin (petechiae, ecchymoses) and mucous membranes (bleeding) may be present.

Medical management

The goal of treatment is to achieve remission or to control the symptoms. Treatment is aimed at eradicating the leukemia with chemotherapy or bone marrow transplant. Combination chemotherapy is the mainstay for treating leukemia. Multiple drugs are used to (1) decrease drug resistance, (2) minimize the drug toxicity by using multiple drugs with varying toxicities (with lower dosages of each), and (3) interrupt cell growth at multiple points in the cell cycle. Observation for drug toxicity is imperative (Table 47.2).

Tremendous progress in the treatment of leukemia has been made in recent years with the use of a complex combination of chemotherapeutic drugs and radiation therapy. Bone marrow transplantation and HSCT may be the treatment of choice in patients with suitable donors and initial remission of the acute leukemia.

In chronic leukemia, which occurs almost exclusively in adults and develops slowly, the desired objectives of treatment depend on the kind of cells involved. Medications commonly used include chlorambucil, hydroxyurea, corticosteroids, and cyclophosphamide. Lymph nodes often are irradiated, and a blood transfusion may be given if anemia is severe. Although medications are not curative in chronic leukemia, they help to prolong life (see Table 47.2).

Nursing interventions and patient teaching

Prevent infection by teaching the patient and family the appropriate precautions for the neutropenic patient and the avoidance of infectious agents. Leukopenia (an abnormal decrease in the number of WBCs to less than 5000 cells/mm³) can be fatal. The usual inflammatory process to control infection is decreased; thus frequent observation for signs and symptoms of infection is necessary. Thrombocytopenia-induced hemorrhage may be life threatening; prevent this condition through safe, gentle care. Control pain through pharmacologic and nonpharmacologic measures. Coping mechanisms may be strained because of pain, complexities of treatment, side effects and toxicities, change in body image, or fear of death. Support the patient and family by developing a positive nurse-patient-family relationship and referring them to community support groups.

Palliative care of the dying child should allow the child and family to experience the best quality of life possible. The child’s illness and palliative care affect the whole family. Therefore the pediatric nurse needs to establish and develop effective communication with the family and between the dying child, parents, and siblings to help allay unnecessary fears and distress about imminent death and to support anticipatory grieving. Varied communication methods should be used to enable the nurse to understand the dying child and siblings’ thoughts and feelings. Drawings can communicate perceptions and emotions about death and dying. These can relate to anger, fear, loss, hope, and acceptance of dying and can assist the nurse in providing holistic care for the child and family. Facilitating creative activity satisfies a basic need and gives the dying child the chance to do something while respecting individual autonomy, choice, and control (National Cancer Institute, 2020).

Table 47.2

Medications for Blood and Lymphatic Disorders
Generic Name	Action	Side Effects	Nursing Implications
cyanocobalamin, vitamin B₁₂	Needed for adequate nerve functioning, protein and carbohydrate metabolism, normal growth, RBC development, and cell reproduction	Flushing, diarrhea, itching, rash, hypokalemia	Assess GI functions and potassium levels at beginning of treatment; stress need for patients with pernicious anemia to return for monthly injections; give intramuscularly only
desmopressin acetate	Promotes reabsorption of water by kidneys and increase in plasma factor VIII levels, which increases platelet aggregation, resulting in vasopressor effect	Nasal irritation, congestion, drowsiness, headache, flushing, nausea, abdominal cramps, heartburn, vulval pain, hypertension	Avoid overhydration; assess pulse and blood pressure when giving drug subcutaneously; monitor factor VIII antigen levels and aPTT
ferrous sulfate	Replaces iron stores needed for RBC development	Nausea, constipation, epigastric pain, black and red tarry stools, vomiting, diarrhea, discolored urine, staining of teeth	Between-meal dosing is preferable but can be given with some foods, although absorption may be decreased; give tablets with orange juice to promote iron absorption; to avoid staining teeth, give elixir iron preparations through straw; oral iron may turn stools black
filgrastim, G-CSF	Stimulates proliferation and differentiation of neutrophils	Fever, alopecia, skeletal pain, nausea, vomiting, diarrhea, mucositis, anorexia	Monitor CBC and platelet count before treatment and twice weekly; refrigerate but do not freeze; avoid shaking; store at room temperature for at least 6 h; discard any vial that has been at room temperature for more than 6 h
folic acid, B complex vitamin	Needed for erythropoiesis; increases RBC, WBC, and platelet formation in megaloblastic anemias	Pruritus, rash, general malaise, bronchospasm, slight flushing	Drug may be administered by deep intramuscular, subcutaneous, or intravenous route; do not mix with other medications in same syringe for intramuscular injections
iron dextran	Released into the plasma and carried by transferrin to the bone marrow, where it is incorporated into Hgb	Stained skin at site of injection, fever, chills, headache, sweating, discolored urine, diarrhea	Administer 0.5-mL test dose by preferred route before therapy; wait at least 1 h before giving remaining portion

Palliative care supports the patient and family’s goals for the future (including their hopes for cure or life prolongation) as well as their hopes for peace and dignity throughout the course of illness, the dying process, and death. The aims of palliative care include guiding the patient and family in making decisions that enable them to work toward their goals during their remaining time. Nurses facilitate shared decision making between health care professionals and patient/family, management of pain and symptoms, recognition and support of grief, and appropriate hospice referrals.

From a physical care perspective, it is challenging to make astute assessments and plan care to help the patient survive the severe side effects of chemotherapy. The life-threatening results of bone marrow suppression (anemia, thrombocytopenia, neutropenia) require aggressive nursing interventions. Additional complications of chemotherapy may affect the patient’s GI tract, nutritional status, skin and mucosa, cardiopulmonary status, liver, kidneys, and neurologic system.

Understand all drugs being administered, including the mechanism of action, purpose, routes of administration, usual doses, potential side effects, safe handling considerations, and toxic effects. In addition, recognize laboratory data reflecting the effects of the drugs. Patient survival and comfort during aggressive chemotherapy are affected significantly by the quality of nursing intervention.

Include the patient and family by discussing procedures, meaning of treatments, and care plans. Be certain to cover the nature of the disease and previous information given to the patient. Community resources for support and information are invaluable for educating the patient and the family. Examine expectations of physical abilities, remission, and future plans. Encourage continuation of the medical regimen and avoidance of situations in which infection can be transmitted. Most patients should receive the pneumococcal vaccine at diagnosis and every 5 years and an annual influenza vaccine (Lyengar & Shimanovsky, 2020). Encourage six to eight small meals a day that consist of a diet high in calories, protein, and vitamins, as well as soft, bland food to reduce irritation to the mouth. Inform patient about the common side effects of their antileukemic therapy and the importance of taking these and other medications on schedule.

Prognosis

The prognosis for patients with AML is dependent on the age of the onset of the disease. The 5-year survival rate for adults is approximately 25%, and 65% for children and adolescents younger than 15 years of age (Nursing Care Plan 47.1). The prognosis for patients with acute lymphocytic leukemia (ALL) is about a 92% chance of experiencing remission in children, whereas adults have about a 69% 5-year survival rate. Adult patients with CLL generally have a 5-year survival rate of 83%. For adult patients with CML, the prognosis depends on the age of the patient, the stage of the disease, and the treatments used. The overall 5-year survival rate is approximately 59% (Lyengar & Shimanovsky, 2020).

Disorders Associated With Platelets

Thrombocytopenia

Etiology and pathophysiology

Thrombocytopenia is an abnormal hematologic condition in which the number of platelets is reduced to fewer than 150,000/mm³. Platelets help maintain a homeostatic balance between bleeding and clotting. When platelet numbers start to fall, this homeostatic balance is disturbed, causing bleeding to occur. The lower the platelet counts, the higher the risk of spontaneous bleeding. The circulating volume of platelets may be affected because there is decreased production, increased destruction, or increased consumption of platelets. Multiple disorders can result in decreased production, including aplastic anemia, leukemia, tumors, and chemotherapy. Platelet destruction can occur as a result of diseases such as Hepatitis B and C, HIV, viral infections, and medications (Box 47.4). Platelets can also be destroyed in the spleen when the spleen is enlarged and traps too many blood cells. Immune disorders such as lupus and arthritis can also result in a destruction of platelets. Increased platelet consumption is caused by disseminated intravascular coagulation (DIC; discussed later). Thrombocytopenia commonly occurs due to a defined cause such as any number of diseases and medications. It may even occur in the presence of ethylenediaminetetraacetic acid (EDTA), which is a preservative used when collecting blood for a CBC, in which case it is pseudothrombocytopenia, and the blood test needs to be repeated in a tube containing citrate instead of EDTA. Thrombocytopenia can rarely occur in isolation from any of the causes mentioned and unrelated to any other immune disorders. This is termed ITP and occurs in the spleen, which is the site of antiplatelet antibody production and platelet clearance (Chaturvedi, Arnold, & McCrae, 2018). ITP is characterized by a destruction of platelets due to an antibody-antigen reaction where antiplatelet antibodies bind platelets. The antibody-bound platelets are then destroyed. The body attempts to replace the destroyed platelets by producing more, but often supply cannot keep up with demand. In addition, the antibodies enter the bone marrow and destroy platelets in the marrow, further reducing supply.

Clinical manifestations

Often patients with thrombocytopenia have no symptoms, and a low platelet finding occurs incidentally. Symptoms rarely occur at platelet counts greater than 50,000/mm³. Patients experiencing platelet counts less than 50,000/mm³ may present with petechiae and purpura. As the count drops even lower, the risk for bleeding from mucous membranes and in cutaneous sites and internal organs increases. Significant risk for serious bleeding occurs once the count is less than 20,000/mm³. Patients exhibit nasal and gingival bleeding, excessive menstrual bleeding, and any injury causes severe bleeding. When the platelet count is less than 5000/mm³, spontaneous, potentially fatal CNS or GI hemorrhage can occur.

Case studies have documented cyclic thrombocytopenia wherein platelet counts cycle from low to high in sync with the menstrual cycle causing menorrhagia (Dogara, Sani, Waziri, et al., 2016).

Assessment

Collection of subjective data includes questioning the patient about recent viral infections (which may produce a transient thrombocytopenia), medications in current use, and the extent of alcohol ingestion.

Nursing Care Plan 47.1 The Patient With Leukemia

Ms. M. is a 26-year-old patient diagnosed with acute lymphocytic leukemia. She is married and the mother of a 3-year-old daughter. Ms. M. has been receiving chemotherapy and is immunocompromised, with a differential white blood cell (WBC) count revealing a neutrophil count of 22%. Her Hgb is 8.8 g/dL, and her platelets are 55,000/mm³. Her mouth appears edematous, and she complains of oral tenderness.

Patient Problem

Potential for Infection, related to leukopenia

Patient Goals and Expected Outcomes	Nursing Interventions	Evaluation
Patient or caregiver will identify measures to prevent or control infection	Inspect all body sites for infection at least daily; note and report fever, sore throat, purulent exudate, chills, cough, burning with urination, erythema, edema, tenderness, and pain.	Patient will remain free of infection.
Patient or caregiver will verbalize and report signs and symptoms of infection	Monitor vital signs. Obtain cultures as ordered. Monitor WBC counts and culture reports. Administer antibiotics on time as ordered. Promote and maintain hygiene integrity of skin and mucous membranes. Use aseptic technique in treatments. Teach the patient and family: • Necessity of avoiding crowds or people with infections while WBC count is decreased. • Personal hygiene measures. • Signs and symptoms of infection.	Patient demonstrates no signs or symptoms of infection; temperature and WBC count are within normal range.

Patient Problem

Impaired Coping, related to diagnosis and disease process

Patient Goals and Expected Outcomes	Nursing Intervention	Evaluation
Patient and family will demonstrate measures to effectively cope by verbalizing role of family, significant others, and support groups in therapeutic coping	Assess coping capabilities of patient and significant others. Discuss disease process and expectations. Alleviate knowledge deficit. Encourage questions and self-expression; listen actively, demonstrate compassion, reassure with touch and personal contact. Assess fear of threat of death; allow time for personal expression and provide one-on-one discussion opportunity.	Patient and family express factors that are causing anxiety and powerlessness.

Critical Thinking Questions

1. What should the nurse do if a visitor with an obvious upper respiratory tract infection is seen approaching Ms. M.’s room?

2. What nursing interventions would be most appropriate in providing therapeutic oral hygiene for Ms. M.?

3. What personal hygiene and activities of daily living would be most beneficial for Ms. M.?

Collection of objective data includes observing the patient’s skin for petechiae and ecchymoses, or possibly hematoma. Epistaxis and gingival bleeding are common. Signs of increased intracranial pressure caused by cerebral hemorrhage may be detected. If bleeding occurs in the GI tract, the patient may vomit blood (hematemesis) or may pass blood in the stool, resulting in bright red blood in the stools from bleeding in the lower GI tract, or dark, tarry stools (melena) from bleeding higher in the GI tract.

Diagnostic tests

To ascertain the characteristics of all blood cells, laboratory studies include platelet count, peripheral blood smear, and bleeding time (BT). In addition, a bone marrow analysis is performed to determine the presence of immature platelets. Examination also reveals the presence or absence of primary bone marrow abnormalities, such as neoplastic invasion or aplastic anemia.

Medical management

By treating the underlying cause of the thrombocytopenia, the patient’s signs and symptoms may improve. Corticosteroids may help if the disorder is related to an autoimmune problem. Packed RBC or platelet transfusions are sometimes necessary. Other medical treatments for ITP include monoclonal antibodies such as Rituximab (Chaturvedi, Arnold, & McCrae, 2018) and thrombopoietin receptor agonists such as eltrombopag and romiplostim (Ghanima, Cooper, Rodeghiero, et al., 2019). Splenectomy is a treatment of last resort if the patient doesn’t respond to monoclonal antibodies or thrombopoietin receptor agonist or if the patient is in the advanced stages of disease (Chaturvedi, Arnold, & McCrae, 2018).

Nursing interventions and patient teaching

Support the medical treatment regimen using specific interventions for specific disease causes. If medication toxicity is the cause, the medication is discontinued. Prevent infections by meticulous asepsis, universal precautions, and gentle handling of the patient. Closely monitor plasma and platelet infusion and whole blood transfusions for reaction and effects on the patient’s condition.

A patient problem and interventions for the patient with thrombocytopenia include but are not limited to the following:

Patient Problem	Nursing Interventions
Compromised Blood Flow to Tissues (cerebral, cardiopulmonary, renal, gastrointestinal, peripheral), related to bleeding	Monitor vital signs and neurologic status Monitor platelet count and abnormal bleeding times Check for bleeding in urine, stool, and emesis Monitor invasive diagnostic procedure sites for bleeding Reduce medical interventions requiring punctures Maintain comfort measures and bed rest Avoid trauma and infection Monitor intake and output for untoward signs Monitor potential sites of hemorrhage

Patient Problem

Nursing Interventions

Compromised Blood Flow to Tissues (cerebral, cardiopulmonary, renal, gastrointestinal, peripheral), related to bleeding

Monitor vital signs and neurologic status

Monitor platelet count and abnormal bleeding times

Check for bleeding in urine, stool, and emesis

Monitor invasive diagnostic procedure sites for bleeding

Reduce medical interventions requiring punctures

Maintain comfort measures and bed rest

Avoid trauma and infection

Monitor intake and output for untoward signs

Monitor potential sites of hemorrhage

The patient must understand the disease process and causative agents to provide self-care and to prevent trauma or infection. Provide instructions on signs, symptoms, and preventive measures: avoid trauma, use stool softeners, maintain a high-fiber diet to prevent constipation, check for the presence of blood, use a soft toothbrush, and blow the nose gently. Stress the importance of notifying the health care provider of signs and symptoms of bleeding.

Prognosis

The prognosis is variable, depending on the underlying cause. Medication-induced thrombocytopenia usually resolves in 1 to 2 weeks after the medication has been stopped. In ITP, treatment may be necessary for 3 to 4 weeks before a complete response is seen. More than 80% of children with immune ITP require no treatment and have a spontaneous recovery from the disorder within weeks after diagnosis. In chronic ITP, transient remissions occur. Approximately 80% of patients benefit from splenectomy immediately after surgery, and 50%-70% experience a complete or partial remission (Chaturvedi, Arnold, & McCrae, 2018).

Disorders Associated With Clotting Factor Defects

Hemophilia

Etiology and pathophysiology

Hemophilia is a coagulation disorder that occurs due to a reduction or deficiency in clotting factors VIII, IX, and XI. Patients missing clotting factor VIII are diagnosed with hemophilia A, those missing clotting factor IX are diagnosed with hemophilia B, and a deficiency in clotting factor XI is diagnosed as hemophilia C. Hemophilia A is the most prevalent, occurring in 80% to 85% of the hemophilia patient population. Hemophilia C is rare and occurs in 1 in 100,000 live births. Worldwide, the number of people living with hemophilia is 400,000 with a higher prevalence in cultures that marry within the family (Mehta & Reddivari, 2020).

Hemophilia is a disease that occurs due to genetic mutations occurring on over 1000 genes that code for clotting factors that exist on chromosome X (Mehta & Reddivari, 2020). As the mutation occurs on chromosome X and the disease is recessive, females are usually asymptomatic carriers for hemophilia while males manifest the disease. It is possible for females to manifest the disease if they inherit an affected X chromosome from the father and asymptomatic carrier X chromosome from the mother, but these instances are rare. In addition, it is estimated that 30% of hemophilia cases occur due to spontaneous genetic mutations (Mehta & Reddivari, 2020). The reduction or absence of clotting factors results in a disruption in the clotting mechanisms of the body, which then leads to prolonged bleeding. The severity of bleeding is dependent on the percentage of normal factor activity present.

Clinical manifestations

The overarching symptom of hemophilia is prolonged bleeding. Hemophilia can be distinguished into mild, moderate, and severe. Patients that retain 5% to 40% of normal factor activity have mild hemophilia and often present with significant bleeding after trauma or surgery. Spontaneous bleeding is generally not present in patients with mild hemophilia. Moderate hemophilia occurs when patients retain 1% to 5% of normal factor activity. These patients experience bleeding after trauma, injury, dental work, or surgery. Though bleeding in joint spaces occurs with severe hemophilia, it does also occur in 25% of the cases in patients with moderate hemophilia. Severe hemophilia occurs when patients retain less than 1% of normal factor activity (Mehta & Reddivari, 2020). These patients bleed spontaneously with extensive, deep bruising (ecchymosis) of muscle tissue, which may show deformity; hemorrhage also occurs into joints, which eventually become ankylosed (i.e., fused or obliterated). Hemarthrosis, or bleeding into a joint space, is a hallmark of severe disease and usually occurs in the knees, ankles, elbows, shoulders, and hips. Pain, edema, erythema, and fever accompany hemarthrosis. Small cuts can prove fatal; blood loss from simple dental procedures may be significant. Urine and stool may reveal bleeding along with unusual bleeding from something as simple as vaccinations (Mayo Clinic, 2020a). Any organ may exhibit bleeding and patients will manifest symptoms accordingly. Patients with mild or moderate hemophilia may not be diagnosed until later, and the age of diagnosis may coincide with the level of precipitating trauma. Patients with severe hemophilia may experience recurrent bleeding even in utero (Mehta & Reddivari, 2020).

Assessment

Subjective data include reports by patient and family of incidents of ecchymosis and hemorrhage from even the slightest trauma. Pain is associated with joint motion.

Collection of objective data includes noting blood in subcutaneous tissues, urine, or stool and noting edematous or immobile joints.

Diagnostic tests

Families with known hemophiliacs may opt to genetically test by chorionic villous sampling, amniocentesis, or obtaining a sample of umbilical cord blood at birth. Patients with high suspicion due to history and physical can diagnose using blood tests including CBC, PT, PTT, and BT. In patients with hemophilia A and B, PTT will be prolonged by two to three times the normal range, whereas PT and BT will be normal. Next, a mixing study will be conducted that will normalize PTT time by contributing clotting factors to correct the deficiency. This test is followed by a factor VIII and IX assay and then genetic testing as confirmation (Mehta & Reddivari, 2020).

Medical management

The treatment of a patient with hemophilia revolves around prophylaxis and management of acute bleeds. Patients can be placed on a prophylactic treatment of factor replacement, which has the advantage of reducing bleeding episodes, which reduces the need for hospitalizations, improves quality of life, and requires less frequent monitoring. Prophylactic treatments significantly reduce the amount of bleeding in joints and therefore reduce hemarthrosis, chronic pain, ankylosis, and the need for joint surgeries. Prophylactic treatments also reduce the risk of developing antibodies to factor replacement. Patients can opt for continuous or intermittent prophylactic treatment. Continuous treatment is defined by infusions of weekly factor replacement for 45 out of the 52 weeks in a year. Prophylactic treatment is also defined as primary, secondary, or tertiary based on the level of disease progress. Prophylactic treatment involves factor VIII or IX replacement two to three times a week.

Management of acute bleeding episodes needs simultaneous management of the location and severity of the bleed and immediate initiation of high-dose clotting factor concentrate (CFC) with factor VIII or IX. For example, intracranial bleeding would require surgical intervention for the cranial bleed as well as CFC. Pain is managed with acetaminophen, Cox-2 inhibitors, and opioids. Aspirin and all NSAIDs are avoided to prevent further bleeding. All venipunctures and injections must be avoided if possible. Desmopressin (DDVAP), a synthetic analog of vasopressin, is given intranasally and induces the release of von Willebrand factor (vWF), which increases endogenous levels of factor VIII three- to fivefold. It can only be used for hemophilia A, as it has no effect on factor IX (Mehta & Reddivari, 2020).

Nursing interventions and patient teaching

Assess the patient’s level of understanding of the clinical course of the disease and prevention of complications. Educate the patient and the entire family because many people may be involved in the patient care. Control hemorrhages in emergency situations by applying pressure and cold to the site. Support and reassurance are imperative. Monitor transfusions of factor VIII concentrate. Supportive care measures include pain management and genetic counseling. Do not give hemophilia patients aspirin or aspirin products, because they can further complicate the bleeding tendency.

Patient problems and interventions for the patient with hemophilia include but are not limited to the following:

Patient Problem	Nursing Interventions
Knowledge deficit, related to long-term illness	Educate patient and family about disease process Promote healthier life choices such as participation in noncontact sports such as swimming, cycling, or table tennis Wear protective gear when exercising Take warm baths to promote relaxation and mobility Wear a medical bracelet
Inadequate Fluid Volume, related to bleeding Compromised Blood Flow to Tissue, related to blood loss from coagulation deficit	Assess for extent of hemorrhage Prevent further hemorrhage or extension Monitor vital signs and laboratory reports Apply cold compresses to bleeding areas Assess for anxiety, shock, disorientation Assess for decreased urinary output Teach safety precautions to prevent trauma Administer analgesia as ordered Move patient gently and slowly, supporting joints Prevent deformity through support, splints, and physical therapy

Patient Problem

Nursing Interventions

Knowledge deficit, related to long-term illness

Educate patient and family about disease process

Promote healthier life choices such as participation in noncontact sports such as swimming, cycling, or table tennis

Wear protective gear when exercising

Take warm baths to promote relaxation and mobility

Wear a medical bracelet

Inadequate Fluid Volume, related to bleeding

Compromised Blood Flow to Tissue, related to blood loss from coagulation deficit

Assess for extent of hemorrhage

Prevent further hemorrhage or extension

Monitor vital signs and laboratory reports

Apply cold compresses to bleeding areas

Assess for anxiety, shock, disorientation

Assess for decreased urinary output

Teach safety precautions to prevent trauma

Administer analgesia as ordered

Move patient gently and slowly, supporting joints

Prevent deformity through support, splints, and physical therapy

Discuss with the patient ways to avoid injury and control bleeding. Also discuss physical activity within limits. Encourage the patient to wear a medical-alert tag. Emergency care teaching includes immobilizing the affected part, applying ice, and notifying the health care provider. Discuss diet to prevent obesity, which puts excess pressure on joints. Regular dental care and preventive dental and medical measures are important aspects. Overprotection is sometimes a factor to discuss. Neither aspirin nor any other medication, including over-the-counter medications, should be taken except with the health care provider’s knowledge (see the Home Care Considerations box).

Home Care Considerations

Hemophilia

• Home management is a primary consideration for a patient with hemophilia because the disease follows a chronic, progressive course.

• The quantity and length of life may be affected significantly by the patient’s knowledge of the illness and understanding of how to live with it.

• Refer the patient and family to the local chapter of the National Hemophilia Foundation to encourage association with other individuals who are dealing with the problems associated with hemophilia.

• Teach the patient with hemophilia to recognize disease-related problems and to learn which problems can be resolved at home and which require hospitalization.

• Immediate medical attention is required for severe pain or edema of a muscle or joint that restricts movement or inhibits sleep and for a head injury, edema in the neck or mouth, abdominal pain, hematuria, melena, and skin wounds in need of suturing.

• Oral hygiene must be performed gently, without trauma.

• Aspirin and aspirin products should not be taken, because they decrease platelet aggregation.

• Understanding how to prevent injuries is an important consideration. The patient can learn to participate in noncontact sports (e.g., golf) and wear gloves when doing household chores to prevent cuts or abrasions from knives, hammers, and other tools.

• The patient should wear a medical-alert tag to ensure that health care providers know about the hemophilia in case of an accident.

• A person with hemophilia who is mature enough, or a family member, can be taught to administer some of the factor replacement therapies at home.

Prognosis

Prior to factor replacement therapies, hemophiliacs had a life expectancy of 11 years and usually died in adolescence. The advent of factor replacement therapy has significantly changed the course of hemophilia in patients. Most patients with hemophilia live normal lives in the absence of comorbid conditions.

von Willebrand Disease

Etiology and pathophysiology

von Willebrand disease (vWD) is an inherited genetic bleeding disorder passed down by either parent or both. In rare cases, vWF can occur due to a spontaneous genetic mutation and even rarer is the possibility of acquired vWF that occurs later in life. The acquired vWF usually occurs due to some other underlying medical condition. It is characterized by abnormally slow coagulation of blood and spontaneous episodes of GI bleeding, epistaxis, and gingival bleeding. The disease is caused by a deficiency of vWF, which is a protein that is critical for platelet adhesion (one of the earliest steps in blood coagulation). Because vWF is bound to factor VIII, protecting it from rapid breakdown in the blood, a patient with vWD also has low factor VIII levels. vWD is the most common bleeding disorder, occurring in 1 out of every 100 people. It occurs equally in men and women, though women tend to notice the symptoms caused by vWD due to heavier than normal menstrual periods and increased bleeding after giving birth (CDC, 2020).

Researchers have identified many variations of vWD, but most fall into three types (CDC.gov, 2020):

• Type 1: Most common and mildest form occurring in 85% of vWD cases. Patient has low levels of vWF and may also have low levels of factor VIII (which is different from hemophilia where factor VIII is very low or absent and vWF levels are normal)

• Type 2: The body produces enough vWF but it is somehow deficient in construction and thus unable to attach to platelet cells. Depending on the abnormality, patients may be classified as having type 2a or type 2b, type 2M, and 2N.

• Type 3: Rarest type occurring in about 3% of cases. Patients have very little or no vWF and have low levels of factor VIII.

Clinical manifestations

The symptoms experienced by patients vary in degree and severity based on the type of vWD. Common symptoms include frequent and hard-to-stop nose bleeds, easy bruising with very little or no trauma, heavy menstrual bleeding that often leads to a diagnosis of anemia, longer than normal bleeding after dental work or giving birth, blood in urine or stool, and painful joints due to bleeding in joint spaces.

Assessment

Subjective data includes history of easy bruising and bleeding, nose bleeds five or more times a year, and reports of blood clotting taking longer than usual.

Objective data includes physical evidence of bruising and bleeding, painful and swollen joints, and signs of anemia.

Diagnostic tests

A CBC will indicate decrease in RBCs, Hgb, and HCT. Prothrombin time (PT) and activated partial thromboplastin time (aPTT) results will be abnormally high as clotting is taking longer. Blood tests for clotting factors will help diagnose vWD and include vWF antigen, vWF activity, Factor VIII clotting activity, and vWF multimers (Mayo Clinic, 2019a).

Medical management

Patients with mild forms of vWD are treated with nasal or IV desmopressin acetate.

This drug is a synthetic form of the human antidiuretic hormone, vasopressin. It causes an increase in vWF release from storage sites in the body and of factor VIII. Desmopressin is often administered prophylactically to patients with mild vWD who require surgery or dental extractions. For patients with more severe forms of vWD or those that don’t respond to desmopressin treatment includes IV transfusions of Recombinant (an organism, cell or genetic material formed by recombining things) VWF and medicines that contain both vWF and factor VIII such as Humate, Wilate, Alphanate, and Koate. Antifibrinolytic drugs such as Amicar and Lysteda can be prescribed to help slow the breakdown of clots and women with vWD can be prescribed birth control pills as it increases the levels of vWF and factor VIII while having the benefit of decreasing heavy menstrual flow (CDC, 2020).

Observation and nursing interventions for hemophilia A and B can be adapted easily to vWD.

Prognosis

The prognosis is usually good for patients with early diagnosis and effective treatment. Most people who have type 1 vWD are able to live normal lives with only mild bleeding issues. Individuals with type 2 are at an increased risk of experiencing mild to moderate bleeding and complications. During times of infection, surgery, or pregnancy the patient often has increased bleeding. Type 3 poses an increased risk for severe bleeding, externally and internally. These patients have a normal life expectancy (Pollak, 2017).

Disseminated Intravascular Coagulation

Etiology and pathophysiology

Disseminated intravascular coagulation (DIC) is a grave but rare coagulopathy resulting from the overstimulation of clotting and anticlotting processes that always occur due to an underlying medical condition involving some type of infection, inflammation, or cancer. Examples of medical conditions that cause DIC include septicemia, obstetric complications (abruptio placentae and preeclampsia or HELLP syndrome), malignancies, tissue trauma, transfusion reaction, burns, shock, and snake bites (Box 47.5). In DIC, widespread clotting (thrombosis) within small vessels occurs, with subsequent damage to multiple organs. Due to widespread coagulation, clotting factors are depleted from other parts of the body thus resulting in an imbalance between clotting and bleeding. The imbalance results in widespread bleeding. Thus, patients in DIC could be clotting and bleeding simultaneously. DIC can be acute or chronic and may develop slowly, over hours or days, or quickly.

Box 47.5 Disorders Usually Associated With Disseminated Intravascular Coagulation

Acute Disseminated Intravascular Coagulation

Chronic Disseminated Intravascular Coagulation

Clinical Manifestations

Clinical manifestations include those of the underlying disorder that causes DIC as well as signs of clotting and bleeding. Blood clots resulting in DVT would cause pain, redness, swelling; blood clots in the microvasculature of the kidney would result in kidney failure and anuria, pain, and hypertension; neurological clots could cause obtunded mental status, coma, and paresthesias; pulmonary system blood clots would result in dyspnea, central cyanosis, palpitations and chest pain; and GI blood clots result in swelling and passing of clots in stool. Bleeding would occur from mucous membranes, venipuncture sites, surgical sites, and all orifices leading to circulatory collapse (hypotension, tachycardia) and acute respiratory distress (central cyanosis, air hunger). The patient exhibits petechiae on the soft palate, trunk, and extremities and bruising especially from venipuncture sites.

Assessment

Subjective data include patient complaints based on sites of bleeding and clotting.

Collection of objective data includes observing for occult or obvious bleeding. Purpura on the chest and abdomen, reflecting fibrin deposits in capillaries, is a common first sign of DIC. Note the color of skin and mucosa and the presence of petechiae. Abdominal tenderness may be present. GI bleeding, hematuria, pulmonary edema, PE, hypotension, tachycardia, absence of peripheral pulses, decreased blood pressure, restlessness, confusion, seizures, or coma may be present.

Diagnostic tests

DIC can be difficult to diagnose especially in chronic cases. Diagnosis is made by reviewing the clinical picture along with laboratory results. The coagulation profile shows prolonged prothrombin and aPTTs, as well as prolonged BT. The platelet count is low, showing marked thrombocytopenia. Other tests show fibrinogen deficiency (hypofibrinogenemia; reflecting the consumption of fibrinogen in DIC) and deficits of factors V, VII, VIII, X, and XII.

D-dimer test results are elevated as d-dimer is a breakdown of fibrinogen and therefore an indirect measure of clotting.

Medical management

The underlying cause of DIC must be addressed and corrected and the symptoms of DIC are treated with supportive medications. Hypovolemia (Box 47.1) caused due to hemorrhage is treated with transfusions of packed RBCs and fluids. Administration of platelet transfusion and factor replacement therapy is initiated when extensive bleeding occurs. FFP may be transfused to replace clotting factors to stop bleeding. Heparin therapy may be ordered if the patient has widespread fibrin deposition without significant bleeding; it is mostly used in cases of chronic DIC.

Nursing Interventions and patient teaching

Protection from bleeding and trauma and application of pressure to sites of hemorrhage are essential nursing measures. Support and reassurance of the patient may aid in relieving high stress levels. Monitor the patient in a quiet, nonstressful environment. Make sure the patient’s bed has padded side rails and use foam or cotton swabs for mouth care. Monitor vital signs and administer heparin, blood and FFP transfusions, and factor replacement therapy as ordered. Use the blood pressure cuff infrequently to avoid subcutaneous bleeding.

Patient problems and interventions for the patient with DIC include but are not limited to the following:

Patient Problem	Nursing Interventions
Potential for Injury, Bleeding, and Fluid Deficit, related to: • Depleted coagulation factors • Adverse effect of heparin (excess heparin, insufficient heparin)	Monitor Hct and Hgb Assess skin surface for signs of bleeding; note petechiae; purpura; hematomas; oozing of blood from IV sites, drains, and wounds; and bleeding from mucous membranes Observe for signs of bleeding from GI and genitourinary tracts Note any hemoptysis or blood obtained during suctioning Monitor level of consciousness (LOC); institute neurologic checklist (mental status changes may occur with the decreased fluid volume or with decreasing Hgb) Monitor vital signs for signs of hemorrhage Observe for signs of orthostatic hypotension (drop of >15 mm Hg when changing from supine to sitting position indicates reduced circulating fluids) Avoid intramuscular injections; any needlestick is a potential bleeding site Apply pressure to bleeding site Prevent trauma to catheter and tubes by proper taping, minimum pulling

Patient Problem

Nursing Interventions

Potential for Injury, Bleeding, and Fluid Deficit, related to:

• Depleted coagulation factors

• Adverse effect of heparin (excess heparin, insufficient heparin)

Monitor Hct and Hgb

Assess skin surface for signs of bleeding; note petechiae; purpura; hematomas; oozing of blood from IV sites, drains, and wounds; and bleeding from mucous membranes

Observe for signs of bleeding from GI and genitourinary tracts

Note any hemoptysis or blood obtained during suctioning

Monitor level of consciousness (LOC); institute neurologic checklist (mental status changes may occur with the decreased fluid volume or with decreasing Hgb)

Monitor vital signs for signs of hemorrhage

Observe for signs of orthostatic hypotension (drop of >15 mm Hg when changing from supine to sitting position indicates reduced circulating fluids)

Avoid intramuscular injections; any needlestick is a potential bleeding site

Apply pressure to bleeding site

Prevent trauma to catheter and tubes by proper taping, minimum pulling

Discuss with the patient and family the signs and symptoms of DIC and have them repeat this information to the nurse or health care provider. Teach the patient to self-administer heparin therapy subcutaneously if prescribed. Instruct the patient and family to avoid mechanical trauma, such as from a hard toothbrush, blade razor, rough nose blowing, or contact sports.

Prognosis

Mortality rates from DIC vary, depending on severity. Death is usually a result of either uncontrolled hemorrhage, irreversible end-organ damage, or both.

Disorders Associated With Plasma Cells

Multiple Myeloma

Etiology and pathophysiology

Multiple myeloma, or plasma cell myeloma, is a malignant neoplastic disease of the bone marrow. Neoplastic plasma cells build up in the bone marrow and produce one or more tumors. The tumors destroy bone (osseous) tissue, especially in flat bones, causing pain, fractures, and skeletal deformities.

The specific immunoglobulin produced by the myeloma cells is present in the blood and/or urine and is referred to as monoclonal protein, M protein, or paraprotein. This protein is a helpful marker to monitor the extent of the disease and the patient’s response to treatment. It is measured by serum or urine protein electrophoresis.

The older adult patient whose chief complaint is back pain and who has elevated total serum protein should be evaluated for possible multiple myeloma. It most frequently occurs in patients at a median age of 70 years and the male to female ratio for occurrence is three to two (Albagoush & Azevedo, 2020). Onset is gradual and insidious; the disease often goes unrecognized for years while the individual experiences frequent, recurrent bacterial infections (the result of immune dysfunction: there is too much of a particular nonfunctional immunoglobulin and not enough normal immunoglobulin). Early detection can decrease the amount of pain and disability resulting from bone destruction and pathologic fractures (Herget, Wäsch, Klein, et al., 2020). 1.8% of all new cancer cases in the United States is diagnosed as multiple myeloma (Albagoush & Azevedo, 2020).

Clinical manifestations

The disease process shows a proliferation of malignant plasma cells and development of single or multiple bone marrow tumors. This is followed by bone destruction with dissemination into lymph nodes, liver, spleen, and kidneys.

The skeletal system symptoms typically involve the ribs, spine, and pelvis. Osteolytic lesions are seen in the skull, vertebrae, and ribs. Vertebral destruction can lead to collapse of vertebrae with ensuing compression of the spinal cord. Patients complain of bone pain that increases with movement. About 30% develop pathologic fractures accompanied by severe pain.

In an individual with multiple myeloma, production of erythrocytes, platelets, and leukocytes is disrupted because the marrow is crowded by the abnormal proliferation of plasma cells. This leads to infection, anemia, and increased potential for bleeding. Calcium and phosphorus drain from bones, leading to hypercalcemia and renal problems. In addition, cell destruction contributes to the development of hyperuricemia (high uric acid in the blood), which, along with the high protein levels caused by the myeloma protein, can result in renal failure.

Assessment

Collection of subjective data includes assessment of the patient’s complaints of pain, especially skeletal pain in the back, pelvis, the spine, and the ribs.

Collection of objective data includes assessing the patient’s facial expression for signs of increased pain with movement, the ability to perform ADLs, increased body temperature, increased potential for bleeding, changes in urine characteristics, and effectiveness of medication administration.

Diagnostic tests

Diagnosis of multiple myeloma is made with radiographic skeletal studies, bone marrow biopsy, and laboratory examination of blood and urine. A monoclonal antibody (M protein) may be present, as evidenced in serum or urine electrophoresis. Bony degeneration also causes loss of calcium in the bones, eventually causing hypercalcemia. Pancytopenia, hypercalcemia, hyperuricemia, and elevated creatinine may be found. In addition, an abnormal globulin known as Bence Jones protein is found in the urine and can result in renal failure.

Radiographic skeletal examinations reveal widespread demineralization, lytic lesions, and osteoporosis. Lytic lesions (destruction of an area of bone due to a disease process) may be seen on bone roentgenograms but are not well visualized on bone scans. Bone marrow studies reveal large numbers of immature plasma cells, which normally account for only 5% of marrow population.

Medical management

Treatments for multiple myeloma can be placed in two categories: patients eligible for bone marrow transplants and patients ineligible for bone marrow transplants. Transplant eligibility is determined based on disease progress and patient functional status. Those patients who are eligible undergo chemotherapy to decrease the size of the tumor, followed by harvesting of stem cells via PBSC transplant and autologous transplant. Patients ineligible for bone marrow transplants receive drug therapies including lenalidomide and dexamethasone, bortezomib and dexamethasone, melphalan/prednisone/bortezomib, or other bortezomib-based regimens. They can also be treated with proteasome inhibitors (stop enzyme complexes, proteasomes, in cells from breaking down proteins important for keeping cell division under control) and monoclonal antibodies (Albagoush & Azevedo, 2020).

Hypercalcemia (resulting from bone destruction) and pain also should be addressed. Analgesics, orthopedic supports, and localized radiation help reduce the skeletal pain. Hospitalization to administer chemotherapy, corticosteroids, and fluids may be required.

Nursing interventions and patient teaching

Care of the patient with multiple myeloma focuses on relieving pain, preventing infection and bone injury, administering chemotherapy and radiation, and maintaining hydration (Box 47.6). Encourage ambulation and adequate hydration to treat hypercalcemia, dehydration, and potential renal damage. Fluid intake of 3 to 4 L/day is encouraged to prevent dehydration and maintain a urinary output of 1.5 to 2 L/day. Patients with multiple myeloma with high tumor burdens (the total mass of tumor tissue carried by a patient with a malignancy) who receive chemotherapy have increased cell lysis and release of uric acid, resulting in hyperuricemia. Weight-bearing helps the bones reabsorb some calcium, and fluids dilute calcium and prevent protein precipitates from causing renal tubular obstruction.

Box 47.6 Chemotherapy Side Effects

Because of the potential for pathologic fractures, be careful when moving and ambulating the patient. A slight twist or strain on the bones may be sufficient to cause a fracture. Attention to the psychosocial, emotional, and spiritual needs is also extremely important.

Patient problems and interventions for the patient with multiple myeloma include but are not limited to the following:

Patient Problem	Nursing Interventions
Potential for Injury, related to: • Osteoporosis • Lytic lesions	Protect from bone injury; use logroll, turning sheet
	Use pillows to support bony prominences
Recent Onset of Pain, related to disease process	Administer analgesics as ordered (such as nonsteroidal anti-inflammatory drugs, acetaminophen, or an acetaminophen-opioid combination). Combination drugs may be more effective than opioids alone in diminishing bone pain Provide comfort measures Assess contributing factors

Teach the patient how to avoid traumatic bone injury and infection. Discuss the importance of adequate hydration and review the pain control modalities available. It is also important to identify spiritual resources. Address the patient’s understanding of the disease, verbalization of discouragement and hopelessness, and desires for emotional and spiritual support.

Prognosis

The prognosis for multiple myeloma is dependent on several factors, including stage when diagnosed, effectiveness of medical treatment, comorbidities, and age of the patient. Generally, older patients with comorbid conditions have poorer survival rates than younger patients. Overall 5-year survival rate is around 35% (Albagoush & Azevedo, 2020).

Disorders of The Lymphatic System

Lymphangitis

Etiology and Pathophysiology

Lymphangitis can occur due to inflammation or infection. Infection typically results in one or more lymphatic vessels or channels from an acute streptococcal infection of the skin and less often staphylococcal infection. The appearance of lymphangitis signals a worsening skin infection that could result in complications such as septicemia. Lymphangitis from inflammatory processes usually occur due to the presence of malignancy.

Clinical Manifestations

Lymphangitis is characterized by tender red streaks that radiate from the site of the infection toward the closest lymph glands. The lymph nodes in the surrounding area are enlarged and tender. Patients experience systemic symptoms such as malaise, fever, chills, headache, muscle aches, loss of appetite, and pain the infected area.

Assessment and diagnostic test

Subjective data includes presence of red streaks, reports of pain and tenderness in the affected area, complaints of headache and muscle pain.

Objective data includes fever, chills, red streaks, swollen and tender lymph nodes. Diagnosis is based on symptoms, presence of skin infections, inflammation due to malignancy, and possible biopsy of the lymph node. Blood cultures may be conducted as sepsis is a complication of lymphangitis.

Medical Management

Administration of antimicrobial drugs by oral or IV route controls the infection. Anti-inflammatory medications may be ordered to reduce inflammation and anti-pyretics for fever. Warm moist compresses may be used to reduce inflammation and pain. In certain cases, surgical management may be needed to remove obstructed or abscessed nodes.

Nursing Interventions

The affected area should be kept clean to promote healing. Rest and extremity elevation may relieve the pressure and reduce any swelling that may have occurred. Patients are taught to report increasing pain, growing streaks, or pus or fluid coming out of the lymph node.

Prognosis

With treatment, the prognosis is usually good.

Lymphedema

Etiology and Pathophysiology

Lymphedema is a primary or secondary disorder characterized by the accumulation of protein rich fluid in the soft tissue that leads to subsequent inflammation, fibrosis, and hypertrophy of adipose tissue (Kim, Hwang, Bae, et al., 2019). The accumulation of lymph fluid in soft tissue can be caused by various conditions. Obstruction of a lymph vessel could occur due to the presence of a cancerous tumor that blocks the movement of fluid into the lymph vessels. Often during cancer treatment, the cancerous tumor plus surrounding affected lymph nodes and vessels are removed which results in a buildup of fluid in soft tissue. Infections occurring in the surrounding tissues can cause lymphedema by damaging lymph vessels. Lymphedema can also occur due to genetic mutations. Lymphedema typically occurs in the arms and legs though it can occur anywhere in the body including face, neck, trunk, abdomen, and genitals. Patients need to understand that lymphedema is a chronic condition that can become severe and cause serious problems.

Clinical Manifestations

Edema experienced by patients can range from mild to severe. Mild edema may cause achy pain, slight heaviness, and restlessness in the extremity. As the edema worsens, the limb feels heavier, patients experience tingling, the skin feels tight and looks thicker and more leathery. As the condition worsens, the patient experiences more restricted range of motion, constant aching and discomfort, and recurring infections. Wounds in the swollen extremity heal slower and joints of the affected extremity are stiff and sore.

Assessment and Diagnostic Test

Subjective data include history of cancer treatment, trauma or damage to the area, and complaints of pain, pressure, and presence of edema.

Collection of objective data includes observation of the extremities for edema, palpation of peripheral pulses, restricted range of motion, and infections in the affected extremity. Patients with recent history of tumor removals are diagnosed based on signs and symptoms. Patients with lymphedema without obvious cause undergo a procedure known as lymphoscintigraphy. This test involves injection of a radioactive substance under the skin between the first and second fingers or toes. If the affected extremity is the left arm, then the right arm is also injected with radioactive dye and studied as a comparison. The patients are then asked to squeeze a rubber ball to allow for transport of the radioactive substances (Kim, Hwang, Bae, et al., 2019). Blockages then can be detected by scanning the flow of the dye through the lymph system.

Medical Management

Treatment for lymphedema focuses on reducing swelling and controlling the pain. A lymphedema therapist can teach patients gentle exercises that encourage fluid drainage. Wrapping or compression bandages compress the affected extremity and move fluid away from the digits. The bandaging should be tightest at the fingers and looser moving up the affected extremity. Manual lymph massage conducted by a specially trained therapist may encourage the movement of lymph fluid. A pneumatic compression sleeve worn over the affected extremity inflates and deflates intermittently helping the fluid to move away from the digits. Mechanical management includes special massage techniques referred to as manual lymph drainage. These techniques should be performed only by specially trained individuals (Mayo Clinic, 2017).

Nursing Interventions and Patient Teaching

The primary goal of care is to increase lymphatic drainage and avoid trauma. Elevation of the extremities while asleep and periodically during the day facilitates drainage. Massage techniques previously discussed help improve lymph drainage. If the patient tolerates light exercise, it should be encouraged. Advise patients to avoid constrictive clothing, shoes, or stockings (except elastic stockings). Patients with lymphedema are at risk for infection in the affected extremity. Good skin care, avoiding injury, inspecting the skin daily for cuts or cracks in the skin, is important. Lotions can be applied to prevent dryness of the skin.

Emotional support for the patient is also important. Address body image disturbance related to the appearance of the lymphedematous extremity. Emphasize that lymphedema need not prevent the individual from engaging in routine activity.

A patient problem and interventions for lymphedema include but are not limited to the following:

Patient Problem	Nursing Interventions
Potential for infection, related to altered lymphatic drainage causing stasis of fluid	Elevate affected extremity Consider physical therapy or range-of-motion exercises (aids lymphatic flow) Assess skin for signs of infection (redness, warmth) Teach application of supportive stockings or elastic sleeves Monitor patient for systemic signs of infection (elevated white blood cell count, increased body temperature, increased erythrocyte sedimentation rate)

Make certain the patient is aware of the condition’s progression and cause. If the disorder is long term and ongoing, discuss how to cope with its effects. Explain the rationale behind nursing interventions to enhance the ongoing medical regimen. Encourage the patient to maintain interests and socialize to enhance feelings of well-being.

Prognosis

Lymphedema has no cure, but signs and symptoms of the condition can be controlled by compliance with treatment. The goal of care is to prevent lymphedema when possible and to initiate prompt treatment when it does occur. Compliance with suggested treatment modalities can prevent complications of the disorder which include lymphangitis, cellulitis, and lymphangiosarcoma (Kim, Hwang, Bae, et al., 2019).

Hodgkin Lymphoma

Etiology and Pathophysiology

Hodgkin lymphoma, previously known as Hodgkin disease, is characterized by painless, progressive enlargement of lymphoid tissue. It is slightly more common in men than in women and accounts for 0.5% of newly diagnosed cancer cases in the United States with a death rate of 0.2% (Shanbhag & Ambinder, 2018). The age of incidence curve is bimodal (two separate populations), with a peak early in life at 15 to 35 years, and a peak later in life at 50 years. The two peaks in incidence may represent separate diseases. The first incident peak suggests a viral cause. Beginning as an inflammatory or infectious process, the condition develops into a neoplasm.

Hodgkin lymphoma has no major risk factors, but the disease occurs more frequently in people who have had mononucleosis (an infection caused by Epstein-Barr virus), have acquired or congenital immunodeficiency syndromes, are taking immunosuppressive drugs after organ transplantation, have been exposed to occupational toxins, or have a genetic predisposition. The presence of HIV increases the incidence of Hodgkin lymphoma.

Lymphoid tissue enlargement usually is noticed first in the cervical nodes and is characterized by abnormal or atypical cells. Reed-Sternberg cells are atypical histiocytes consisting of large, abnormal, multinucleated cells in the lymph nodes found in Hodgkin lymphoma. These cells increase in number, replacing normal cells. The main diagnostic feature of Hodgkin lymphoma is the presence of Reed-Sternberg cells in lymph node biopsy specimens.

The disease is believed to arise in a single location (the lymph nodes in most patients) and then spread along adjacent lymphatics. It eventually infiltrates other organs, especially the lungs, spleen, and liver. In the majority of patients, the cervical lymph nodes are affected first. Unless they exert pressure on adjacent nerves, the enlarged nodes are not painful. When the disease begins above the diaphragm, it remains confined to lymph nodes for a variable period. Disease originating below the diaphragm frequently spreads to extralymphoid sites such as the liver.

Clinical Manifestations

Painless enlargement of the cervical, axillary, or inguinal lymph nodes is most often the initial development. Anorexia, unexplained rapid weight loss, fever, night sweats, fatigue, and pruritus are complaints associated with this condition. One other unusual symptom is pain in the lymph nodes after ingesting alcohol (Mayo Clinic, 2019b). Night sweats, weight loss, and fever, which are referred to as “B” symptoms, are associated with a worse prognosis (Box 47.7). Low-grade fever may occur. Anemia and leukocytosis follow, with development of respiratory tract infections.

Assessment

Subjective data include the common complaints of fatigue and appetite loss. Pruritus is often severe. After the ingestion of even small amounts of alcohol, individuals with Hodgkin lymphoma may complain of a rapid onset of pain at the site of the disease. The cause for the alcohol-induced pain is unknown. Bone pain occurs later in the disease’s course.

Box 47.7 Cotswold-Modified Ann Arbor Staging System for Hodgkin Lymphoma

Stage I: Disease affecting a single lymph node region or lymphoid structure (e.g., spleen, thymus, Waldeyer ring).

Stage II: Disease affecting two or more discrete lymph node regions confined to the same side of the diaphragm.

Stage III: Disease affecting two or more discrete lymph node regions or lymphoid structures on both sides of the diaphragm.

Stage IV: Disease that has spread to one or more extranodal sites (that does not meet the criteria for E) or an extralymphatic structure including involvement of the bone marrow, liver, or lungs.

Designations

A: Absence of B symptoms ^a
B: Presence of B symptoms ^a
S: Involvement of the spleen

E: Single extranodal site or involvement of an extranodal site that is contiguous to an involved nodal region.

X: Bulky disease as defined as >1/3 mediastinum at its widest part or a nodal mass >10 cm at its greatest diameter.

From Hoffman R, Anastasi J, Weitz J, et al: Hematology: Basic principles and practice, ed 7, St. Louis, 2018, Elsevier.

Collection of objective data includes palpating enlarged cervical and supraclavicular lymph nodes. Splenomegaly, hepatomegaly, and abdominal tenderness are found. Excoriation of skin and evidence of scratching from pruritus are noted. Clinical signs and symptoms vary depending on where the enlarged lymph nodes are located. If Hodgkin lymphoma affects lymph nodes inside the chest, the swelling of these nodes may press on the trachea, stimulating a cough reflex or dyspnea. Some patients may complain of pain behind the sternum and difficulty swallowing (ACS, 2018a).

Diagnostic Tests

Peripheral blood studies show anemia (normocytic, normochromic), WBC increase, and an abnormal erythrocyte sedimentation rate. Other blood studies may show hypoferremia (low iron level in the blood) caused by excessive iron intake by the liver and the spleen, elevated leukocyte alkaline phosphatase from liver and bone involvement, hypercalcemia from bone involvement, and hypoalbuminemia from liver involvement. Chest radiographic examination may reveal a mediastinal mass. CT or MRI can detect retroperitoneal node involvement. Lymph node biopsy that includes laparoscopy for retroperitoneal nodes is performed. Bone marrow biopsy is an important aspect of staging. A CT scan and an ultrasound examination can indicate an enlarged spleen or liver. The presence of Reed-Sternberg cells remains a hallmark of Hodgkin lymphoma.

Positron emission tomography (PET), CT scans, bone scans, and bone marrow biopsy aid in the staging of the disease by determining the extent of any metastasis (ACS, 2018a). The liver, lungs, and bone are common areas of metastasis.

Medical Management

Treatment depends on the staging process (see Box 47.7). The stage of Hodgkin lymphoma must be established before selecting an appropriate treatment plan.

Combination chemotherapy is used in some early stages in patients believed to have resistant disease or to be at high risk for relapse. Chemotherapy and radiation therapy are used against the generalized forms (stages III and IV). Advances in treatment now enable some stage IIIB and stage IV diseases to be cured with high-dose chemotherapy and bone marrow or peripheral stem cell transplantation (SCT). The site of the disease and the amount of resistant disease after chemotherapy determine the role of radiation in supplementing chemotherapy.

Treatment of early-stage Hodgkin lymphoma consists of two to four cycles of ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) chemotherapy.

For advanced-stage Hodgkin lymphoma some people have ABVD for up to eight cycles. Other possible combinations include Stanford V (mechlorethamine, doxorubicin, vinblastine, vincristine, bleomycin, etoposide, and prednisone), and BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone) (ACS, 2017a). If chemotherapy does not work well or the lymphoma comes back, some people have high-dose chemotherapy with SCT (ACS, 2018a).

Nursing Interventions and Patient Teaching

Plan care according to the staging level. Awareness of side effects of radiation therapy or chemotherapy is important in preparing the patient to deal effectively with the treatment. Because the survival of patients with Hodgkin lymphoma depends on their response to treatment, helping the patient deal with the consequences of treatment is extremely important. Comfort measures focus on skin integrity. Soothing baths with an antipruritic medication (as prescribed) can be effective. Control fever and moisture with medication (with attention to increased fluid intake) and linen changes as necessary to prevent further skin problems. Explain extensive tests to the patient to aid in reduction of anxiety and difficulty coping.

Patient problems include Anxiousness and Fearfulness, related to unknown outcome, and Potential for Infection, related to compromised immune system. An additional patient problem and interventions for the patient with Hodgkin lymphoma include but are not limited to the following:

Patient Problem	Nursing Interventions
Compromised Skin Integrity, related to: • Pruritus • Jaundice • Diaphoresis	Assess skin and level of discomfort Administer skin care by baths and keep patient clean and dry Apply calamine lotion, cornstarch, sodium bicarbonate, and medicated powders to relieve pruritus Maintain adequate humidity and a cool room to decrease pruritus Monitor vital signs for fever; assess for perspiration and change linen, keeping it wrinkle free

Understanding the disease through education and teaching is important for the patient to perform self-care and retain independence. Fertility issues may be of particular concern because this disease frequently is seen in adolescents and young adults. Help ensure that these issues are addressed soon after diagnosis. The effect on the patient’s life, as well as on significant others, is a prime consideration in patient attitude and adjustment. Realistic approaches to the illness and therapies are imperative. Referrals for patients seeking counseling for stress management can be helpful. Discuss special nutritional considerations concerning excess weight loss or an undernourished condition.

Prognosis

The staging of the disease is important in the prognosis. The disease is classified as stage I if the cancer is found in only one lymph node area or lymphoid organ, such as the thymus, or the cancer is found only in one area of a single organ outside the lymph system. Stage II is characterized by cancer being found in two or more lymph node areas on the same side of the diaphragm, or the cancer extends locally from one lymph node area into a nearby organ. Stage III is determined by the presence of cancer in lymph node areas on both sides of the diaphragm, or the cancer is in lymph nodes above the diaphragm and in the spleen. Cancer that has spread widely into at least one organ outside of the lymph system, such as the liver, bone marrow, or lungs is classified as stage IV Hodgkin lymphoma (ACS, 2018a). In general, the earlier the stage of the cancer, the higher the survival rate and chance for a cure. The 5-year survival rate for Hodgkin lymphoma is not based on staging directly but on whether the cancer is localized, reginal, or distant. Patients with localized Hodgkin lymphoma have a 5-year survival rate of 92%, regional Hodgkin lymphoma has a 5-year survival rate of 94%, and distant has a 5-year survival rate of 78% (ACS, 2018a).

Non-Hodgkin Lymphoma

Etiology and Pathophysiology

Non-Hodgkin lymphoma (NHL) is a neoplasm originating in lymphoid tissues of the body. Neoplasms are of B-cell or T-cell origin and give rise to cancerous cells that are varied in terms of epidemiology, etiology, clinical features, and response to therapy (Sapkota & Shaikh, 2020). NHL can also be classified as indolent or aggressive. Indolent NHL tends to grow and spread slowly, and patients with this type of NHL may be treated with a “watch and wait” approach. Aggressive NHL tends to grow and spread quickly and requires immediate treatment. The risk factors for getting NHL include the following: individuals over the age of 60; men more than women; more common in Caucasians than African Americans and Asians; occurs in developed countries more so than third-world countries; more common in people exposed to chemicals such as benzene, herbicides, insecticides, and chemotherapy drugs; immunosuppressed and HIV; autoimmune diseases; and infections such as Epstein Barr virus and herpes (ACS, 2018b).

Clinical Manifestations

Common symptoms of NHL include enlarged lymph nodes, fever, sweating and chills, weight loss, pruritus, fatigue, and susceptibility to infection. Abdominal distension may result if lymph nodes of the abdomen, liver, or spleen are enlarged as a result of the disease process. If NHL is in the chest, the patient may experience pain in the chest, pressure, a cough, and shortness of breath (ACS, 2018b). There is no hallmark pathologic feature in NHL that parallels the Reed-Sternberg cell of Hodgkin lymphoma. However, all NHLs involve lymphocytes arrested in various stages of development.

Assessment

Subjective data include frequent patient complaints of fatigue, malaise, and anorexia.

Collection of objective data includes examination of the abdomen for splenomegaly. Enlarged lymph nodes are also evident. Fever, night sweats, and weight loss are usually present.

Diagnostic Tests

Biopsies of lymph nodes, liver, and bone marrow are performed to establish the cell type and pattern. A bone scan may reveal fractures, lesions, and tumor infiltration. PET scans, CT, MRI, and chest x-ray are common tests for diagnosing NHL. Common serum lab tests include the CBC, immunohistochemistry (detects specific antibodies), flow cytometry (identifies specific types of cells), and DNA/genetic testing. Diagnostic studies used for NHL resemble those used for Hodgkin lymphoma (ACS, 2018b).

Staging, as described for Hodgkin lymphoma, is used to guide therapy. The four-stage system used with Hodgkin lymphoma is the same system used for NHL.

Medical Management

Once the diagnosis is made, the extent of the disease (staging) is determined. Accurate staging is crucial to determine the treatment regimen. The therapeutic regimen for NHLs includes chemotherapy and radiation. Indolent (slow-growing) lymphomas have a naturally long course but are difficult to treat effectively. In contrast, more aggressive lymphomas are more likely to be cured because they are more responsive to treatment. Some chemotherapy agents used are cyclophosphamide, vincristine, prednisone, doxorubicin, bleomycin, and methotrexate. The monoclonal antibody rituximab was approved for the treatment of follicular lymphoma. Ibritumomab is another monoclonal antibody that can be used in patients who are refractory to rituximab, or in conjunction with it. Conventional chemotherapy used to treat patients with relapsed, aggressive NHL, who are still responding to salvage chemotherapy, is not as effective as high-dose chemotherapy with autologous (tissue derived from the same individual) HSCT (ACS, 2018b).

Patients with lymphoma commonly receive radiation to the chest wall, mediastinum, axillae, and neck—the region known as the “mantle field.” Some patients also need radiation to the abdomen; paraaortic area; spleen; and, less commonly, the pelvis.

Chemotherapy remains the traditional method of treatment of NHLs that are not localized. High-dose chemotherapy with PBSC or bone marrow transplantation may be indicated. Interferon is being used to help in boosting the immune system. Monoclonal antibodies are being designed to attack a specific target, such as a substance on the surface of lymphocytes, and destroy cancer cells. Targeted cancer therapies such as the use of monoclonal antibodies promise to be more selective for cancer cells than normal cells, thus harming fewer normal cells, reducing side effects, and improving quality of life (ACS, 2018b).

Nursing Interventions and Patient Teaching

Supportive care of the patient during radiation and chemotherapy is primary in nursing management. Observation for complications follows. Further intervention is similar to that for Hodgkin lymphoma.

Explanations of the extensive diagnostic workup and its importance for staging the disease and determining the treatment plan are an important focus of patient teaching during the diagnostic period.

Prognosis

The 5-year survival rate for NHL is 73% if local, 72% if regional, and 55% if distant (ACS, 2018a). Patients with aggressive lymphomas have worse prognosis, whereas patients with low-grade lymphomas survive 6 to 10 years. Low-grade lymphomas can turn into aggressive lymphomas (Sapkota & Shaikh, 2020).

Nursing Process For The Patient With A Blood or Lymphatic Disorder

Assessment

Collect data from diverse sources: patient and family observation, physical examination, and diagnostic evaluation results (see Table 47.1).

The subjective data collected at the onset of the disease process are generally vague and nonspecific: malaise, fatigue, and weakness. The patient may relate a history of illness, easy bruising, bleeding tendencies with petechiae, and ecchymoses. Integumentary changes (including pruritus, nonhealing cuts and bruises, draining lesions, jaundice, and palpable subcutaneous nodules) may be reported. Edema and tenderness in lymph node regions may be accompanied by pain, sometimes severe. GI complaints are noted, as well as cardiovascular and respiratory changes. Neurologic complaints include headache, numbness, tingling, paresthesias, and behavioral alteration (see the Lifespan Considerations box).

Lifespan Considerations