Chapter 28 - Nursing Care of Patients With Hematologic and Lymphatic Disorders

Disorders of Red Blood Cells: Nursing Process

- Data collection Monitor hemoglobin and hematocrit levels and other laboratory studies as ordered and report any downward trend. - Imbalanced nutrition Instruct the patient with iron deficiency about high-iron foods and the correct use of an iron supplement. An iron supplement should be taken with vitamin C to enhance absorption. Instruct the patient to notify the primary care provider of any side effects related to iron supplements such as nausea, diarrhea, constipation, and dark stools. Administer intramuscular iron injections by the Z-track method to avoid staining the injection site. Administer oral iron 1 hour before or 2 hours after meals to enhance absorption.

Disorders of Red Blood Cells: Etiology

- Dietary deficiencies Iron, folic acid, and vitamin B12 are all essential to the production of healthy RBCs. A deficiency of any of these nutrients can cause anemia. Pernicious anemia is associated with a lack of intrinsic factor in stomach secretions, which is necessary for absorption of vitamin B12. - Hemolysis Hemolysis is the destruction, or lysis, of RBCs. Destruction of RBCs leads to a type of anemia called hemolytic anemia. - Other causes Thalassemia anemia is a hereditary anemia found in persons from Southeast Asia, Africa, Italy, and the Mediterranean islands. do not synthesize hemoglobin normally.

Disorders of Red Blood Cells: Aplastic Anemia

- Pathophysiology Aplastic anemia differs from other types of anemia in that the bone marrow becomes fatty and incapable of producing the needed numbers of RBCs. the cells that are produced are normal in size and shape, but there are not enough of them to sustain life. Left untreated, aplastic anemia is almost always fatal. - Etiology congenital—that is, the person is born with bone marrow incapable of producing the correct number of cells. Or it may be due to exposure to toxic substances such as industrial chemicals (e.g., benzenes and insecticides), chemotherapy medications, or use of cardiopulmonary bypass during surgery. Other causes include certain bacterial and viral infections, such as tuberculosis and hepatitis, or autoimmune disease. - Signs and symptoms vary with the severity of the bone marrow failure. As the disease progresses and the pancytopenia worsens, other symptoms, such as tachycardia and heart failure, may appear. Ecchymoses and petechiae appear on the skin surface because of the reduced platelet count. - Diagnostic tests The diagnosis of aplastic anemia begins with a CBC. The most definitive test is a bone marrow biopsy. Because the bone marrow is essentially dead, the result is often described as a "dry tap" in which pale, fatty, yellow, fibrous bone marrow is extracted instead of the red, gelatinous bone marrow normally seen. total iron-binding capacity (TIBC) and serum iron level. - Therapeutic measures Unfortunately, it is often difficult to determine the cause, and there is no way to reverse the damage already done. Today the most effective treatment for aplastic anemia is bone marrow transplantation. - Nursing management If the patient's platelet count is low (usually less than 20,000), the patient is placed on bleeding precautions. If the WBC count is low, the patient must be protected from infection.

Hemorrhagic Disorders: Disseminated Intravascular Coagulation

- Pathophysiology Disseminated intravascular coagulation (DIC) involves a series of events that result in severe hemorrhage. Organs most often affected include the kidneys and the brain, but other blood-engorged organs, such as the lungs, the pituitary and adrenal glands, and the GI mucosa, are commonly involved. - Etiology DIC can develop after any condition in which the body has sustained major trauma. - Signs and symptoms Abnormal bleeding without a history of a serious hemorrhagic disorder is a cardinal sign of DIC. Early signs of bleeding in- clude petechiae, ecchymoses (Fig. 28.5), and bleeding from venipuncture sites. Bleeding may progress to IV sites, skin tears, surgical sites, incisions, and the GI tract and oral mucosa. Joints become painful and enlarged if bleeding into the joints occurs. - Diagnostic tests Initial laboratory findings in DIC include a prolonged pro- thrombin time (PT) and partial thromboplastin time (PTT), decreased platelet count, and increased evidence of fibrin degradation products.

Disorders of White Blood Cells: Leukemia

- Pathophysiology Leukemia is a malignant disease of the WBCs that affects all age groups. The immature WBCs (blast cells) generate in an explosive fashion in the bone marrow, lymph tissue, and spleen. Areas especially prone to infiltration with immature WBCs are the oral mucosa, anus, sinuses, and lungs.At the time of diagnosis, these areas are often inflamed, painful, and infected. The patient becomes anemic because of the lack of RBC production, and bleeding becomes a problem as fewer and fewer platelets are manufactured. - Classifications • Leukemias are classified as either acute or chronic and either lymphoid or myeloid. Symptoms of the acute leukemias begin suddenly, and the patient is very sick, whereas chronic leukemias develop slowly and patients can be surprised by the diagnosis because they feel well. Lymphoid leukemias affect the lymphocytes. Myeloid leukemias originate in the stem cells of the bone marrow. Acute lymphocytic leukemia (ALL) is the most common cancer in children and involves abnormal growth of the lymphocyte precursors (lymphoblasts). Chronic lymphocytic leukemia (CLL) predominantly affects the B and T lymphocytes and usually occurs in adults. Chronic leukemia usually de velops in a three-phase process. The first, insidious phase is characterized by anemia and mild bleeding abnormalities. Some patients have developed leukemia after being treated for another unrelated malignancy using radiation or chemotherapy. - Signs and symptoms Symptoms are similar for all types of leukemia and include low-grade fever caused by infection and pallor , weakness, lethargy, shortness of breath, and malaise caused by anemia. - Diagnostic tests Although a simple CBC often points to ward the diagnosis, only bone marrow aspiration can show the degree of proliferation of the malignant WBCs and confirm the diagnosis of leukemia. - Therapeutic measures Systemic chemotherapy aims to eradicate the leukemic cells and induce a remission. Remission means that the bone marrow is free to produce normally occurring cells in normal proportions without production of the immature WBCs. Remission is not the same as cure. There are four phases to the treatment of leukemia: induction, intensification, consolidation, and maintenance. In- duction is the period in which an attempt is made to get the patient into remission. This first phase is difficult because chemotherapy is given in very high doses and on an aggressive timetable. The nurse must help the patient deal with anemia, thrombocytopenia, and leukopenia, as well as other side effects. Intensification is similar to the initial in- duction phase, using the same drugs at even higher doses. The next phase, consolidation, is used to ensure that all leuk emic cells have been eradicated from the body. Finally, the patient graduates to maintenance therapy in which the patient is kept free of leukemic cells and in remission for a period of years. Radiation therapy is sometimes used in addition to chemotherapy for initial treatment of leukemia. It may be directed at the entire body or at specific areas where leukemic cells are collecting. Bone marrow transplant (BMT) is sometimes used to treat leukemia. Preparation for BMT includes high-dose chemotherapy and/or total body ir- radiation. The goal is to destroy all of the patient's malignant bone marrow and then, at the last possible moment, replace it with a donor's clean and healthy bone marro w (allogenic transplant). Another type of bone marrow transplant, known as an autologous transplant, uses the patient's own diseased bone marrow, which is harvested, chemically treated and cleaned, stored, and later reinfused.

Disorders of Red Blood Cells: Sickle Cell Anemia

- Pathophysiology Sickle cell anemia is an inherited anemia in which the RBCs have a specific mutation that makes the hemoglobin in the red cells very sensitive to oxygen changes. Any time a decrease in the oxygen tension is sensed, the cells begin an observable physical change from their usual spherical shape to a sickle or crescent shape. The result is congestion, clumping, and clotting. Because the cells are fragile, the life span of the RBCs in patients with sickle cell anemia is significantly decreased. Normal red cells live about 120 days. Sickled cells survive only about 10 to 20 days. - Etiology Sickle cell disease (SCD) is an autosomal recessive hereditary disorder. sickle cell anemia is most often found in those of African or Eastern Mediterranean heritage. Symptoms do not appear in infants until after the age of 6 months because up to that age, the infant is using hemoglobin manufactured during fetal life. - Signs and symptoms The rapid return of the oxygen level to normal usually returns the cells to their normal shape. It tends to collect in the capillaries and veins of chest and abdominal organs, as well as joints and bones, and can cause infarction (tissue necrosis resulting from lack of blood supply). Tissue necrosis causes pain, fever, and swelling. Crises may occur without any apparent cause. In general, crises last from 4 to 6 days. - Diagnostic tests The most telling feature of SCD is a blood smear that shows sickle-shaped RBCs in circulation. Hemoglobin electrophoresis is a test used to determine the presence of hemoglobin (Hgb) S, the abnormal form of hemoglobin. - Therapeutic measures Some patients may be placed on low-dose oral penicillin to help pre vent infections, decreasing the risk of crises. During acute crises, the patient is admitted to the hospital. The nurse can anticipate that the patient will require sedation and analgesia for severe pain and blood transfusions to replace the sickled red cells lost by their being caught, crushed, and destroyed. Deferasirox (Exjade) is a medication that may be given to de- crease the excess iron levels. Corticosteroids may reduce the need for analgesics and oxygen. Hydroxyurea (Droxia) is a drug that has been shown to decrease crises, but it can cause life-threatening side effects. - Nursing management In the patient in crisis, assess circulation in the extremities every 2 hours, including pulse oximetry, capillary refill, peripheral pulses, and temperature. Advise the patient to a void tight- fitting clothing that restricts circulation. avoid strenuous exercise, which increases oxygen demand, as well as cold temperatures and smoking, which cause vaso- constriction. Alcoholic beverages can also trigger a crisis and should be avoided.

Idiopathic Thrombocytopenia Purpura

- Pathophysiology and Etiology Acute idiopathic thrombocytopenic purpura (ITP) results from increased platelet destruction by the immune system. Any time platelet numbers are reduced, the risk for bleeding increases. Acute ITP usually affects children between ages 2 and 6, whereas chronic ITP mainly affects adults over age 60. Acute ITP usually occurs after an acute viral illness such as rubella or chickenpox. Hepatitis C virus and human immunodeficiency virus (HIV) can also be triggers. It may be drug induced or associated with pregnancy. ITP is believed to be related to an immune system dysfunction. - Signs and symptoms ITP produces clinical changes that are common to all forms of thrombocytopenia: petechiae, ecchymoses, and bleeding from the mouth, nose, or GI tract. - Diagnostic tests A platelet count of less than 20,000/mm and a prolonged bleeding time suggest ITP. - Therapeutic measures The goal of treatment is to have an adequate (if not normal) platelet count and no bleeding.

Polycythemia

- Pathophysiology and Etiology Polycythemia is really two separate disorders that are easily recognizable by similar characteristic changes in the RBC count. In both forms of polycythemia, the blood becomes so thick with an overabundance of RBCs. Laboratory tests show a hemoglobin level greater than 18 mg/dL, an RBC mass greater than 6 million, and a hematocrit of more than 55%. Polycythemia vera (PV) is known as primary polycythemia. Most people with PV have a specific genetic mutation. In PV, the RBCs, platelets, and WBCs are all overproduced. PV is usually found in patients over age 50. secondary polycythemia is the result of long- term hypoxia. Secondary polycythemia is a compensatory mechanism rather than an actual disorder. - Signs and Symptoms A patient with PV commonly presents with hypertension, vision changes, headache, vertigo, dizziness, and ringing in the ears (tinnitus). Laboratory results show an increased level of all bone marrow components (RBCs, WBCs, platelets), which is called panmyelosis. bleeding disorder called acquired von Willebrand syndrome. PV are due to the major problems of hypervolemia, hyperviscosity, and engorgement of capillary beds. Without treatment, patients with PV die of thrombosis or hemorrhage. - Diagnostic tests Diagnosis of PV is made based on a CBC and bone marrow aspiration. The bone marrow or blood will also show the genetic mutation if it is present. - Therapeutic measures Treatment of PV takes place in two stages. The first stage is to decrease the hyperviscosity problem. The most common first-line treatment is therapeutic phlebotomy. Phlebotomy involves withdrawal of blood, which is then discarded. From 350 to 500 mL of blood are removed each time on an every- other-day basis, with the goal being a hematocrit of about 45%. Chemotherapeutic agents or radiation therapy, including radioactive phosphorus, may be used to suppress production of blood cells in some patients. - Nursing management Instruct the patient to drink at least 3 L of water daily to re- duce blood viscosity. Encourage smoking cessation, avoid- ance of tight or restrictive clothing, and elevation of feet when resting to prevent impairment of circulation.

Multiple Myeloma: Diagnostic Tests

A CBC shows moderate to severe anemia. The WBC count may show an increase in the number of white cells secondary to infection. Blood and urine studies are positive for M-type globulins (called Bence-Jones proteins when found in the urine) in 40% of patients. X-ray e xaminations or magnetic resonance imaging (MRI) may show changes in the lungs and diffuse osteoporosis in bones not already riddled with holes. Bone marrow biopsy is done to confirm the diagnosis and determine the stage of the disease. C-reactive protein (CRP) is elevated in multiple myeloma. Elevated CRP levels are also associated with increased fatigue.

Disorders of Red Blood Cells: Diagnostic Tests

A complete blood cell count (CBC) is done to determine the number of RBCs and WBCs per cubic millimeter. Hemoglobin and hematocrit levels are below normal in anemia. Patients with pernicious anemia have low gastric acid levels, and many have antibodies to intrinsic factor.

Disorders of Red Blood Cells: Pathopysiology

A decrease in the number of RBCs can be traced to three conditions: (1) impaired production of RBCs, as in aplastic anemia and nutrition deficiencies; (2) increased destruction of RBCs, as in hemolytic or sickle cell anemia; or (3) massive or chronic blood loss.

Non-Hodgkin's Lymphomas

All of the other types of lymphomas are clumped into a diverse classification known as the non-Hodgkin's lymphomas (NHLs). Non-Hodgkin's lymphomas arise in the lymphoid tissues of the body, just as Hodgkin's disease does, but they differ in several ways.

Lymphatic Disorders: Therapeutic Measures

Appropriate therapy includes the use of radiation and chemotherapy and depends on the stage of the disease. Radiation therapy, administered on an outpatient basis o ver a 4- to 6-week period, can cure most patients with stage I or stage II disease. Combinations of chemotherapy and radiation therapy are used for patients with stage III and stage IV disease.

Non-Hodgkin's Lymphomas: Signs and Symptoms

Clinical features of malignant lymphomas include enlarged, painless, rubbery nodes in the cervical and supraclavicular areas, axillae, and groin; enlarged tonsils and adenoids; and occasional symptoms of dyspnea and cough.

Non-Hodgkin's Lymphomas: Diagnostic Tests

Diagnosis is confirmed by histological evaluation of biopsied lymph nodes, tonsils, bone marrow, liver, bowel, skin, or other affected tissues.

Lymphatic Disorders: Diagnostic Tests

Diagnosis usually begins with a lymph node biopsy of the easiest lymph node to access. Lymph node biopsies are done to check for abnormal histiocyte proliferation, nodular fibro- sis, and necrosis. Other tests include bone marrow biopsy and aspiration, liver and spleen biopsies, routine chest x-ray examination, abdominal computed tomography (CT) scan to check for disease in the liver and spleen, lung scan, and bone scan. Lymphangiography may be performed to view the flow of lymph in the lymph netw ork. A gallium scan can also be done to view lymph tissue. Hematologic tests (e.g., CBC) may show wide variability of RBCs, indicating mild to severe anemia. The WBC count is often abnormal and extreme (either very high or very low) because of bone marrow infiltration by disease. These same tests are used for staging the disease into one of four stages: • Stage I disease is limited to a single lymph node or site or a single organ. • Stage II disease occurs when two or more nodes are involved on the same side of the diaphragm. • Stage III disease affects nodes on both sides of the diaphragm. • Stage IV, the most serious form of the disease and the least curable, includes widely disseminated disease in both lymph nodes and other organs such as bone marrow or liver.

Hemophilia

Hemophilia is a group of hereditary bleeding disorders that result from a severe lack of specific clotting factors. The two most common are hemophiliaA (classic hemophilia) and hemophilia B (Christmas disease). Von Willebrand's disease is another related bleeding disorder. - Pathophysiology Hemophilia A accounts for 80% of all types of hemophilia and results from a deficiency of factor VIII. Hemophilia B is a factor IX deficiency; about 15% of people with hemophilia have this type. - Etiology Hemophilia A and B are inherited as X-linked recessive traits. This means that the female carrier (daughter of an affected father) has a 50% chance of transmitting the gene to each son or daughter. - Signs and symptoms Bleeding occurs as a result of injury or, in severe cases, spontaneously (unprovoked by injury). Bleeding into the muscles and joints (hemarthrosis) is common and can cause acute pain. Severe and repeated episodes of joint hemorrhage cause joint deformities, especially in the elbows, knees, and ankles, which decrease the patient's range of motion and ability to walk. The patient with moderate hemophilia has an occasional bout of spontaneous bleeding. In severe hemophilia, spontaneous bleeding occurs more frequently. - Diagnostic tests Laboratory data reveal a prolonged PTT. Once the missing factor is identified, the type of hemophilia is determined and necessary treatments can be implemented. - Therapeutic measures Hemophilia is not curable. Treatment is aimed at pre venting crippling defor- mities and increasing life expectancy and involves stopping bleeding episodes by administering the missing clotting factors. Mild hemophilia A may be treated with injection or nasal inhalation of desmopressin (DDAVP, antidiuretic hor- mone), which can stimulate the body to release more clotting factors. More severe hemophilia A is treated with factor VIII; hemophilia B is treated with factor IX. Blood transfusions are uncommon but may be necessary after severe trauma or surgery.

Lymphatic Disorders: Hodgkin's Disease

Hodgkin's disease is a lymphoma. Its distinguishing feature is the presence of Reed-Sternberg cells, which make it different from all the other forms of lymphoma. Hodgkin's disease is more prevalent in men than in women and occurs most often in young adults ages 15 to 40. Hodgkin's disease is the most curable type even when the disease is widespread at the time of diagnosis.

Multiple Myeloma: Therapeutic Measures

Long-term treatment of multiple myeloma consists of a two- pronged approach: (1) managing the disease and (2) manag- ing the symptoms. To manage the disease, corticosteroids (prednisone or dexamethasone) and oral or IV chemotherapy agents are given. Thalidomide may be given to slow the pro- gression of the disease. Lenalidomide (Revlimid) is chemi- cally similar to thalidomide but has fewer side effects. The goal of drug therapy is to suppress plasma cell proliferation, which then helps decrease the amount and speed of bone destruction. Another medication for multiple myeloma is bortezomib (Velcade).

Lymphatic Disorders: Pathophysiology

Lymph nodes are made of tightly bound fibers and cells that serve as filtering devices for the body's immune system. Most often, Hodgkin's disease begins as a single changed lymph node, usually in the cervical lymph nodes of the neck. The path of cancer infiltration is usually the same as the path of lymph fluid flow. Left untreated, other lymphoid tissues such as the spleen become infiltrated with Hodgkin's disease. A tentative diagnosis of Hodgkin's disease is based on one or more painlessly enlarged nodes in the cervical, axillary, or inguinal areas.

Lymphatic Disorders

Lymphatic disorders include Hodgkin's disease and the non- Hodgkin's lymphomas. Because the spleen is part of the lymph system, this section also discusses splenectomy.

Multiple Myeloma

Multiple myeloma is a deadly cancer of the plasma cells in the bone marrow. When the disease is caught in its early stages, treatment can prolong life by 3 to 5 years. More important, early detection can decrease the amount of pain and disability due to bony destruction and pathological fractures. Multiple myeloma currently has a 45% survival rate at 5 years.

Lymphatic Disorders: Signs and Symptoms

Painless swelling in one or more of the common lymph node chains is a usual presentation. With just a few sips of any type of alcohol-contain- ing beverage (beer, wine, or liquor), the patient may describe intense pain at the site of disease. Because the lymph nodes in the upper chest and neck are often involved, the patient may have symptoms of obstruction, such as cough, dysphagia, or stridor.

Multiple Myeloma: Signs and Symptoms

Skeletal pain is the most common complaint. Other signs and symptoms include achiness of the long bones, joint swelling and tenderness, low-grade fever, and general malaise. The patient may be unable to feel the true temperature of bath water and be burned or may be unable to feel wounds and infections on the feet. Occasionally, patients have pathological fractures of the long bones. Because calcium is mobilized from the bones and into the blood, the patient is at risk for hypercalcemia. Signs and symptoms of hypercalcemia include anorexia, nausea, vomiting, mental changes (especially confusion), seizures, weakness, and fatigue. Kidney stones may result as the e xcess calcium passes through the kidneys. Patients are susceptible to infection because of compromised immune function. Pneumonia is a common finding in patients with multiple myeloma. They may develop anemia because of bone marrow dysfunction and reduced erythropoietin formation by diseased kidneys. Risk for bruising and bleeding occurs due to thrombocytopenia. Patients often develop kidney failure because the filtering capacity of the kidney becomes blocked by calcium. Other factors include recurrent infections and deposits of myeloma cells in the kidneys.

Splenectomy

Splenectomy is the surgical removal of the spleen. This is sometimes used to treat selective hematologic disorders. - Preoperative care Before the surgery, ensure that the CBC and coagulation profile. Vitamin K is often ordered to correct clotting factor deficiencies.

Disorders of Red Blood Cells: Signs and Symptoms

Symptoms of anemia include pallor, tachycardia, tachypnea, irritability, fatigue, and shortness of breath. In addition to these symptoms, the patient with pernicious (vitamin B12) anemia may experience numbness of the hands or feet and weakness because vitamin B12 is needed for normal neurologic function. Pernicious anemia is also associated with a sore, beefy red tongue.

Non-Hodgkin's Lymphomas: Etiology

The cause of NHL is unclear, but some viruses, such as the Epstein-Barr and herpes viruses, are thought to play a role in their development.

Multiple Myeloma: Etiology

The cause of multiple myeloma is unkno wn. Research suggests genetics may be one factor. People who work in rubber, leather, farming, and petroleum industries are more likely to develop multiple myeloma. Obesity, exposure to radiation, and long-term exposure to hair dyes also increase risk.

Lymphatic Disorders: Etiology

The exact cause of Hodgkin's disease is unknown. A possible viral origin has been proposed; it is more common in people who have had mononucleosis. impaired immune function, such as those with acquired immunodeficiency syndrome (AIDS) or those taking immunosuppressant drugs after organ transplant, are also at higher risk.

Non-Hodgkin's Lymphomas: Pathophysiology

The most distinguishing difference is the absence of the Reed- Sternberg cells in NHL.

Splenic Disorders

The spleen is involved in a number of disorders, including cancers of the blood, lymph, and bone marrow; hereditary conditions such as SCD; and acquired problems such as idiopathic thrombocytopenia. If the spleen enlarges markedly, the condition is referred to as splenomegaly.

Non-Hodgkin's Lymphomas: Therapeutic Measures

Treatment usually involves multimodal therapy, including the use of chemotherapy and radiation therapy in combination. Radiation therapy is gi ven to af fected areas in adv anced stages of NHL. Stem cell transplant may be tried in patients with advanced disease.

Disorders of Red Blood Cells: Anemias

anemia describes a condition in which there is a deficiency of RBCs, hemoglobin, or both, in the circulating blood.

Multiple Myeloma: Pathophysiology

this disorder, cancerous plasma cells in the bone marro w begin reproducing uncontrollably. These cells infiltrate bone tis- sue all over the body and produce hundreds of tumors that begin to devour the bone tissue. Multiple myeloma usually affects the bones of the skull, pelvis, ribs, and vertebrae. As the disease progresses, plasma cells infiltrate the major organs, including the liver, spleen, lymph nodes, lungs, adrenal glands, kidneys, skin, and GI tract. Although the overall result of the disease is the devastating destruction of the bone and widespread osteoporosis, death is often from sepsis.

Hematologic Disorders

• When red blood cells (RBCs) are affected, oxygen transport is also affected, causing symptoms related to poor oxygenation. • When white blood cells (WBCs) are affected, the patient is unable to effectively fight infections. • If platelets or clotting factors are affected, bleeding disorders occur..