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Chapter 26 Hematologic Disorders GENERAL OVERVIEW Blood, the body fluid circulating through the heart, arteries, capillaries,

and veins, consists of plasma and cellular components. The average male adult has about 5.5 L of blood; the average female 4.5 L. Plasma, the fluid portion, accounts for 55% of the blood volume and is composed of 92% water, 7% protein, and 1% inorganic salts; nonprotein organic substances such as urea; dissolved gases; hormones; and enzymes. Plasma proteins include albumin, fibrinogen, and globulins. Cellular components include erythrocytes (red blood cells [RBCs]), leukocytes and lymphocytes (white blood cells [WBCs]), and platelets. These cells are derived from pluripotent stem cells in the bone marrow, a process known as hematopoiesis (see Figure 26-1). Under normal conditions, only mature cells are found in circulating blood. The cellular components of blood account for 45% of the blood volume.

FIGURE 26-1 Steps in differentiation of blood cells. CHARACTERISTICS OF CELLULAR COMPONENTS Blood has multiple functions that are carried out by plasma or the cellular components (see Table 26-1). TABLE 26-1 Functions of Blood FUNCTION METHOD CELLS AND SUBSTANCES INVOLVED Oxygen and carbon dioxide transport Binding to hemoglobin; dissolved in plasma Erythrocyte Hemoglobin
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Plasma Plasma proteins Plasma Plasma Plasma Granulocytes Monocytes Lymphocytes Immunoglobulins Other substances Platelets Clotting factors Water Electrolytes

Nutrient and metabolite transport

Bound to plasma proteins; dissolved in plasma In plasma In plasma In plasma to site of infection or foreign body

Hormone transport Transport of waste products to kidneys and liver Transport of cells and substances involved in immune reactions

Clotting at breaks in blood vessels

Hemostasis

Maintenance of fluid balance

Blood volume regulation

Body temperature regulation Maintenance of acid-base balance Erythrocytes (RBCs)


Peripheral vasoconstriction or dilation Acid-base regulation

Electrolytes

Enucleated, biconcave disc. Approximately 5 million erythrocytes per cubic millimeter of blood. Cell contents consist primarily of hemoglobin, essential for oxygen transport. Whole blood contains 14 to 15 g of hemoglobin per 100 mL of blood. Circulate about 115 to 130 days before elimination by reticuloendothelial system, primarily in spleen and liver.

Leukocytes See Table 26-2. TABLE 26-2 Characteristics of White Blood Cells
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CELL Neutrophil Eosinophil

MAJOR FUNCTION PHYSICAL CHARACTERISTICS Ingest and destroy microorganisms (phagocytosis) Small cell, multilobed nucleus, most plentiful leukocyte Host resistance to helminthic infections; also Bilobed nucleus; red-staining granules allergic response Basophil Allergic response Bilobed nucleus; granules containing heparin and histamine Monocyte Phagocytosis Large cell, kidney-shaped nucleus B Produce antibodies (immunoglobulins); humoral Small, agranular lymphocyte immunity T Regulation of immune response; cellular immunity Small, agranular; include cytotoxic, helper (T4), and suppressor (T8) T cells; lymphocyte identified by surface markers

Approximately 5,000 to 10,000 leukocytes (WBCs) per cubic millimeter of blood. Classified as granulocytes or mononuclear leukocytes. o Granulocytes account for about 70% of all WBCs; have abundant granules in cytoplasm; include neutrophils, basophils, and eosinophils. o Mononuclear leukocytes have single-lobed nucleus and granule-free cytoplasm; include monocytes and lymphocytes.

Platelets (Thrombocytes)

Approximately 150,000 to 450,000 platelets per cubic millimeter of blood. Small particles without nuclei arise as a result of budding from giant cells (megakaryocytes) in bone marrow. Primary function is to control bleeding through hemostasis.

ASSESSMENT SUBJECTIVE DATA The patient who presents with a hematologic disorder may have a disruption of the hematologic, immune, or coagulation system, producing a diverse array of symptoms. Patients commonly present with vague complaints of fatigue, frequent infections, swollen glands, and bleeding tendencies. Characterize these complaints and obtain a review of systems, concentrating on the neurologic, respiratory, cardiovascular, GI, genitourinary (GU), and integumentary systems to look for more clues of hematologic dysfunction. REVIEW OF SYSTEMS

Skin and mucous membranes: Any bruises, infections, drainage, or bleeding from wound sites? Neurologic: Any dizziness, tingling or numbness (paresthesia), headache, forgetfulness or confusion, difficulty walking (disturbance in gait), tiredness (fatigue), weakness?
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Respiratory: Experiencing shortness of breath, especially on exertion? Cardiovascular: Chest pain or feelings of funny heart beats in your chest (palpitations)? GI: Any bleeding from your gums, abdominal pain, black stools, or blood-streaked vomit (emesis)? How about any mouth sores, rectal pain, or diarrhea? GU: Describe your menstrual flow. How often do you change pads? For how many days? Any blood in your urine or discomfort on urination?

Key History Questions


What are your present medications? Do you take over-the-counter (OTC) medications, vitamins, herbals, or nutritional supplements? What else have you taken in the past several months? What medical problems have you had in the past? Surgery? Ask specifically about partial or total gastrectomy, splenic injury or splenectomy, tendency to bleed (eg, with dental procedures), infectious diseases, human immunodeficiency virus (HIV) infection, cancer. What is your occupation? Ask about exposure to substances, such as benzene, pesticides, and ionizing radiation. Do you have a family history of hematologic or malignant disorder? Determine the social history and lifestyle. Do you use illicit drugs or alcohol? What is your pattern of sexual activity?

PHYSICAL EXAMINATION Physical examination may produce abnormal assessment findings in various body systems, caused by anemia, uncontrolled bleeding or clotting, or altered immune function that leads to infection in patients with hematologic disorders. Perform a systematic physical examination, paying careful attention to the cardiovascular, respiratory, and integumentary systems. Key Examination Findings

Tachycardia; dyspnea; shiny smooth tongue; ataxia; pallor of conjunctivae, nail beds, lips, and oral mucosa suggest anemia. Decreased blood pressure (BP), altered level of consciousness (LOC), hematuria, tarry stools, petechiae, bleeding sites suggest altered clotting. Fever; tachycardia; abnormal breath sounds; delirium; oral lesions; erythema, swelling, tenderness, and drainage of the skin suggest infection.

DIAGNOSTIC TESTS LABORATORY STUDIES Laboratory studies routinely done for patients with hematologic disorders include complete blood count (CBC), blood smear, and iron profile. Blood samples for these tests are most accurate when obtained by venipuncture (see Procedure Guidelines 26-1). Complete Blood Count Description

Generally includes absolute numbers or percentages of erythrocytes, leukocytes, platelets, hemoglobin, and hematocrit in blood sample.
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Erythrocyte (RBC) indices can be done to provide information on the size, hemoglobin concentration, and hemoglobin weight of an average RBC; aids in diagnosis and classification of anemias. o Leukocyte (WBC) differential can be done to determine the percentage of each type of granulocyte (neutrophils, eosinophils, and basophils) and nongranulocytes (lymphocytes and monocytes). Absolute value of each is determined by multiplying the percentage by the total number of WBC. Used to evaluate infection or potential for infection and identify various types of leukemia.

PROCEDURE GUIDELINES 26-1 Obtaining Blood by Venipuncture EQUIPMENT


70% alcohol Dry sterile gauze pads Vacutainer or syringe 20G needles or equivalent needleless supplies Blood tubes Disposable gloves

PROCEDURE Nursing Action Performance phase 1. Reassure the patient. Explain that relatively little blood will be taken.

Rationale

2. 3. 4. 5.

6. 7.

1. The patient is reassured when the nurse displays selfassurance and competence in relating to people and when performing technical skills. Wash hands and put on gloves. 2. Protects nurse from possible exposure to blood. Instruct the patient to extend arm; the arm should be held straight at the elbow. 3. Exposes antecubital area. Apply the tourniquet directly above the elbow with just sufficient pressure to 4. A tourniquet increases venous pressure and makes the vein prevent venous return. more prominent and easier to enter. Inspect the area to visualize the vein, including the antecubital area, wrist, dorsum 5. Select a vein that is visible, palpable, and well fixed to (back) of hand, and top of foot (if necessary). Palpate the vein. surrounding tissue so that it does not roll away. (Some veins may be deep and can be only palpated.) Clean the skin with alcohol. Allow to dry. 6. Cleaning the skin reduces pathogens. Fix chosen vein with the thumb and draw the skin taut immediately below the site 7. The vein may roll beneath the skin when the needle before inserting needle to stabilize the vein. approaches its outer surface (especially in elderly and extremely thin patients).
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8. Hold the syringe or vacutainer between the thumb and last three fingers with the bevel of the needle up and directly in line with the course of the vein. Insert the needle quickly and smoothly under the skin and into the vein. 9. Insert tube into vacutainer and allow to fill. For syringe, gently pull back on the plunger until desired amount of blood is obtained. 10. Release the tourniquet as soon as specimen is obtained, then remove tube from vacutainer. 11. Withdraw the needle slowly. 12. Apply a dry sterile gauze pad to puncture site and request patient to apply gentle but firm pressure to site for 24 minutes. 13. Make a blood smear from the needle if ordered. 14. If blood was obtained in a syringe, use needleless supplies per facility procedure and gently eject the blood sample into tubes. 15. Invert the tube gently several times to mix blood with anticoagulant, if present. 16. Label specimens correctly and send to laboratory immediately. 17. Dispose needle and syringe in appropriate containers to avoid possible spread of blood-borne viral diseases. Clean spills with 10% bleach solution. Remove gloves and wash hands.

8. A pop may be felt as the needle enters the vein.

9. Use minimal suction on syringe to prevent hemolysis of blood and collapse of the vein.

11. Slow withdrawal of the needle is less painful. 12. Firm pressure over the puncture site prevents leakage of blood into surrounding tissues with subsequent hematoma development. 14. Slowly transfer the blood into the test tube without forming bubbles. 15. For some tests, the blood is allowed to coagulate in the test tube. 16. Specimens should be sent to the laboratory with a minimum of delay for optimum reliability. 17. Bleach will kill any pathogens, such as HIV.

Nursing and Patient Care Considerations Blood sample can be drawn at any time without fasting or patient preparation. Blood Smear Description Blood specimen prepared for microscopic viewing using appropriate stains, allowing visual analysis of numbers and characteristics of cells; can identify abnormal cells of certain anemias, leukemias, and other disorders that affect the blood stream. Nursing and Patient Care Considerations Can be done from blood sample drawn for CBC; no additional sample or patient preparation is necessary. Iron Profile Description Test completed on blood sample that generally includes levels of serum ferritin, iron, total iron-binding capacity, folate, vitamin B2, and is used to determine type and severity of anemia.
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Nursing and Patient Care Considerations Recent administration of chloramphenicol, hormonal contraceptives, iron supplements, and corticotropin (ACTH) may affect results of serum iron and iron-binding capacity. No patient preparation needed. OTHER DIAGNOSTIC PROCEDURES Bone Marrow Aspiration Description

Aspiration of bone marrow from the iliac crest or sternum to obtain specimen to examine microscopically and to perform a biopsy (see Procedure Guidelines 26-2). Purposes include diagnosis of hematologic disorders; monitoring of course of illness and response to treatment; diagnosis of other disorders, such as primary and metastatic tumors, infectious diseases, and certain granulomas; and isolation of bacteria and other pathogens by culture.

Nursing and Patient Care Considerations


Give medication for pain and anxiety before or after the procedure as ordered. A bone marrow aspiration with biopsy is more painful and may require the use of conscious sedation with appropriate monitoring. Watch for bleeding and hematoma formation after procedure.

Lymph Node Biopsy Description


Surgical excision or needle aspiration usually of a superficial lymph node in the cervical, supraclavicular, axillary, or inguinal region. Performed to determine the cause of lymph node enlargement, to distinguish between benign and malignant lymph node tumors, and to stage metastatic carcinoma.

PROCEDURE GUIDELINES 26-2 Bone Marrow Aspiration and Biopsy EQUIPMENT


Bone marrow aspiration tray Marrow aspiration needles with stylets Towels 25G and 22G needles Two 25-mL syringes Three 5-mL syringes
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Local anesthetic (1% procaine or Xylocaine) Sterile gauze squares Sterile gloves, drape Skin antiseptic Masks and protective eyewear for physician and nurse (check your facility's policy) Laboratory equipment Coverslips Microscopic slides Test tubes (plain and heparinized) Scalpel blade and handle

PROCEDURE Nursing Action Rationale Preparatory phase 1. Explain the procedure to the patient. Tell patient when the skin will be marked, antiseptic 1. An explanation helps the patient to cope with applied, and the needle puncture performed. anticipated stress. Tactile sensations (pressure, cold) can be misinterpreted as pain unless the patient is forewarned. 2. Give analgesic or tranquilizer as requested, 30 minutes before procedure. May not be 2. An analgesic or a sedative may minimize pain, necessary for aspiration. discomfort, and anxiety during procedure. 3. Place the patient in prone or supine position. 3. To expose desired site. 4. The following sites are most frequently used: a. Posterior superior iliac crest b. Anterior iliac crest (if patient is very obese) Iliac crest aspiration/biopsy Performance phase (Nurse assists physician) 1. Position the patient on the abdomen (prone) or on side with top knee flexed. 1. a. The posterior iliac crest is located and marked. a. The iliac crest provides a large marrow cavity at the posterior superior iliac spine away from nearby abdominal organs. b. The skin area is prepared and draped. The marked area is infiltrated with local b. Tell the patient to expect a needle prick followed by a anesthetic through the skin and subcutaneous tissue to the periosteum of the bone. burning sensation. The periosteum is the region of greatest sensitivity. c. A small incision may be made. c. The biopsy needle is large, and a small incision facilitates insertion.
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d. The bone marrow needle, with stylet in place, is introduced through the incision.

d. The needle is pointed toward the anterior superior iliac spine and brought into contact with the posterior iliac spine. e. The needle is advanced and rotated by using firm and steady pressure. When the needle e. There is usually decreased resistance when the bone is felt to enter the outer cortex of the bone marrow cavity, the stylet is removed and the marrow cavity is entered. The actual aspiration may syringe attached. Negative pressure is applied, and a small volume of blood and cause brief pain, and the patient should be marrow is aspirated. forewarned. f. A biopsy is taken by using a special needle equipped with a sharp cutting edge and a f. Bone marrow appears rusty-red and normally has a hollow core. thick, fluidlike consistency. g. After removal of needle, apply pressure to site and dressing. g. Prevents bleeding from puncture site. Dressing keeps site clean and dry until healed.

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Nursing and Patient Care Considerations


Local anesthetic is usually given. Specimen is placed in normal saline or 10% formaldehyde solution for transportation to the laboratory for cytologic and histologic evaluation.

GENERAL PROCEDURES AND TREATMENT MODALITIES


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SPLENECTOMY The spleen is a fist-sized organ located in the upper left quadrant of the abdomen. It includes a central white pulp where storage and some proliferation of lymphocytes and other leukocytes occurs, and a peripheral red pulp involved in fetal erythropoiesis, and later in erythrocyte destruction and the conversion of hemoglobin to bilirubin. It may be surgically removed because of trauma or to treat certain hemolytic or malignant disorders with accompanying splenomegaly. A laparoscopic technique may be used to remove a normal to slightly enlarged spleen in benign conditions, such as idiopathic thrombocytopenic purpura, hemolytic anemia, or sickle cell disease. Preoperative Management

For general aspects of preoperative nursing management. Stabilization of preexisting condition: o For trauma: volume replacement with I.V. fluids, evacuation of stomach contents via nasogastric tube to prevent aspiration, urinary catheterization to monitor urine output, assessment for pneumothorax or hemothorax and possible chest tube placement. o For hemolytic or malignant disorder with accompanying thrombocytopenia: coagulation studies, administration of coagulation factors (eg, vitamin K, fresh-frozen plasma, cryoprecipitate), platelet and red cell transfusions. o Preoperative pulmonary evaluation and teaching. o For patient undergoing elective splenectomy, polyvalent pneumococcal vaccine 10 to 14 days before procedure.

Postoperative Management

For general aspects of postoperative nursing management. Prevention of respiratory complications: hypoventilation and limited diaphragmatic movement, atelectasis of left lower lobe, pneumonia, left pleural effusion. Monitoring for hemorrhage. Administration of opioids for pain and observance for adverse effects. Monitoring for fever. o Postsplenectomy fever mild, transient fever is expected. o Persistent fever may indicate subphrenic abscess or hematoma Monitoring daily platelet count: thrombocytosis (elevation of platelet count) may appear a few days after splenectomy and may persist during first 2 weeks.

Potential Complications

Pancreatitis and fistula formation: tail of pancreas is anatomically close to splenic hilum. Hemorrhage. Atelectasis and pneumonia.
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Overwhelming postsplenectomy infection (OPSI)increased risk of developing a life-threatening bacterial infection with encapsulated organisms, such as Streptococcus pneumoniae, Neisseria meningitidis, or Haemophilus influenzae type b. The incidence of OPSI is 0.23% to 0.42% per year with a lifetime risk of 5%. An OPSI is a medical emergency, and requires immediate I.V. antibiotics in an intensive care setting. I.V. immunoglobulins are also used.

NURSING ALERT The risk of OPSI is highest soon after splenectomy and in patients whose splenectomy occurred during childhood or for a malignant disease. Early symptoms include fever and malaise; the infection may progress within hours to sepsis and death, with mortality as high as 50% to 70%. Patient education after splenectomy should include the risks of OPSI, recognition of early symptoms and prompt seeking of medical attention, MedicAlert bracelet, immunization against S. pneumoniae, H. influenzae type b, and N. meningitidis, and in some cases, prophylactic and standby antibiotics. Nursing Diagnoses

Ineffective Breathing Pattern related to pain and guarding of surgical incision Risk for Deficient Fluid Volume related to hemorrhage caused by surgery of highly vascular organ Risk for Injury (thromboembolism) related to thrombocytosis Risk for Infection related to surgical incision and removal of the spleen Acute Pain related to surgical incision

Nursing Interventions Maintaining Effective Breathing


Assess breath sounds and report absent, diminished, or adventitious sounds. Assist with aggressive chest physiotherapy and incentive spirometry. Encourage early and progressive mobilization.

Monitoring for Hemorrhage


Monitor vital signs frequently and as condition warrants. Measure abdominal girth and report abdominal distention. Assess for pain and report increasing pain. Prepare patient for surgical reexploration if bleeding is suspected.
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Avoiding Thromboembolic Complications


Monitor platelet count daily, report abnormal result promptly. If elevated, assess for possible thromboembolism. o Assess skin color, temperature, and pulses. o Advise patient to report chest pain, shortness of breath, pain, or weakness. Report signs of thromboembolism immediately.

Preventing Infection

Assess surgical incision daily or if increased pain, fever, or foul smell. Maintain meticulous hand washing and change dressings using sterile technique. Teach patient to report signs of infection (fever, malaise) immediately. Educate patient and family regarding OPSI, including plan for postsplenectomy immunizations, recognition of symptoms, use of prophylactic and standby antibiotics.

Relieving Pain

Administer opioids or teach self-administration, as prescribed and as necessary to maintain level of comfort. Warn patient of adverse effects, such as nausea and drowsiness; watch for hypotension and decreased respirations. Teach the use of nonpharmacologic methods, such as the use of music, relaxation breathing, progressive muscle relaxation, distraction, and imagery to help to manage pain. Document dosage of medications and response to medication. Make sure patient has analgesics for use postdischarge.

Patient Education and Health Maintenance


Teach care of incision. Encourage to gradually increase activity according to guidelines given by surgeon. Advise proper rest, nutrition, and stress avoidance while recovering from surgery. Encourage follow-up as directed by surgeon and primary care provider to maintain immunizations. Encourage patient to seek prompt medical attention for any infections and to contact health care provider immediately for high fever.

Evaluation: Expected Outcomes

Respirations unlabored, breath sounds clear


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Vital signs stable, abdominal girth unchanged Pulses strong, extremities warm and without pallor or cyanosis Afebrile, no purulent drainage from incision Verbalizes decreased pain

ANEMIAS Anemia is the lack of sufficient circulating hemoglobin to deliver oxygen to tissues. Anemia has multiple causes, and is commonly associated with other diseases and disorders (eg, renal disease, cancer, Crohn's disease, alcoholism). Anemia may be caused by inadequate production of RBCs, abnormal hemolysis and sequestration of RBCs, or blood loss. Iron deficiency anemia, pernicious anemia, folic acid deficiency, and aplastic anemia are the anemias most commonly seen in adults. Treatments for anemia range from nutritional counseling and supplements to RBC transfusions. One biotherapy treatment with significant potential is the administration of exogenous erythropoietin (Epoetin alpha or Darbepoetin alfa), a growth factor stimulating production and maturation or erythrocytes. It is used to stimulate RBC production in anemias associated with chronic renal failure, chemotherapy treatment, and HIV. IRON DEFICIENCY ANEMIA (MICROCYTIC, HYPOCHROMIC) Iron deficiency anemia is a condition in which the total body iron content is decreased below a normal level, affecting hemoglobin synthesis. RBCs appear pale and are small. Pathophysiology and Etiology

The most common cause is chronic blood loss (GI bleeding, excessive menstrual bleeding, hookworm infestation), but anemia may also be caused by insufficient intake of iron (weight loss, inadequate diet), iron malabsorption (small bowel disease, gastroenterostomy), or increased requirements (pregnancy, periods of rapid growth). Decreased hemoglobin may result in insufficient oxygen delivery to body tissues. Iron deficiency anemia, the most common type of anemia, is found in 10% to 30% of all adults in the United States. It is a major health problem in developing countries. Symptoms generally develop when hemoglobin has fallen to less than 11 g/100 ml.

Clinical Manifestations

Headache, dizziness, fatigue, tinnitus Palpitations, dyspnea on exertion, pallor of skin and mucous membranes Smooth, sore tongue; cheilosis (lesions at corners of mouth) Koilonychia (spoon-shaped fingernails) Pica (craving to eat unusual substances)
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Diagnostic Evaluation

CBC and iron profiledecreased hemoglobin, hematocrit, serum iron, and ferritin; elevated red cell distribution width and normal or elevated total iron-binding capacity. Determination of source of chronic blood loss may include sigmoidoscopy, colonoscopy, upper and lower GI studies, stool and urine for occult blood examination.

Management

Correction of chronic blood loss. Oral or parenteral iron therapy. o Oral ferrous sulfate preferred and least expensive; treatment continues until hemoglobin level is normalized and iron stores replaced (up to 6 months). o Parenteral therapy may rarely be used when patient cannot tolerate or is noncompliant with oral therapy. May use iron dextran (Imferon) or iron sorbitex (Jectofer).

Complications

Severe compromise of the oxygen-carrying capacity of the blood may predispose to ischemic organ damage, such as myocardial infarction or cerebrovascular accident. Anaphylaxis to parenteral iron therapy.

Nursing Assessment

Obtain history of symptoms, dietary intake, past history of anemia, possible sources of blood loss. Examine for tachycardia, pallor, dyspnea, and signs of GI or other bleeding.

Nursing Diagnoses

Imbalanced Nutrition: Less Than Body Requirements related to inadequate intake of iron Activity Intolerance related to decreased oxygen-carrying capacity of the blood Ineffective Tissue Perfusion related to decreased oxygen-carrying capacity of the blood

Nursing Interventions Promoting Iron Intake


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Assess diet for inclusion of foods rich in iron. Arrange nutritionist referral as appropriate. Administer iron replacement as ordered. Technique of parenteral iron administration: o Allow small amount of air in syringe and use new 2-inch needle for injection to avoid tracking medication through subcutaneous tissue and resulting painful induration. o Retract skin over muscle of upper outer quadrant of buttock laterally before inserting needle (Z-track technique) to prevent leakage along track and staining of skin. o Inject deeply and slowly into upper outer quadrant of buttock. Wait a few seconds before withdrawing needle. DRUG ALERT

Anaphylactic reactions may occur after parenteral iron administration. Monitor patient closely for hypotension, angioedema, and stridor after injection. Do not administer with oral iron. Oral supplements may stain teeth give with straw. Increasing Activity Tolerance

Assess level of fatigue and normal sleep pattern; determine activities that cause fatigue. Assist in developing a schedule of activity, rest periods, and sleep. Encourage conditioning exercises to increase strength and endurance.

Maximizing Tissue Perfusion


Assess patient for palpitations, chest pain, dizziness, and shortness of breath; minimize activities that cause these symptoms. Elevate head of bed and provide supplemental oxygen as ordered. Monitor vital signs and fluid balance.

Patient Education and Health Maintenance


Educate patient on proper nutrition and good sources of iron: select well-balanced diet that includes animal proteins, iron-fortified cereals and bread, green leafy vegetables, dried fruits, legumes, nuts. Teach patient about iron supplementation. o Take iron on empty stomach, with full glass of water or fruit juice. o Liquid forms may stain teeth; mix well with water or fruit juice and use straw. o Anticipate some epigastric discomfort, change in color of stool to green or black, and in some cases nausea, constipation, or diarrhea. Prevent and treat constipation with increased fiber, fluids, and exercise. Report GI intolerance to health care provider. o Keep iron medications away from children: overdose may be fatal. Encourage follow-up laboratory studies and visits to health care provider.
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Evaluation: Expected Outcomes


Incorporates several foods high in iron into diet; takes prescribed iron supplementation as ordered Tolerates increased activity; obtains sufficient rest Vital signs stable without complaints of chest pain, palpitations, or shortness of breath

MEGALOBLASTIC ANEMIA: PERNICIOUS A megaloblast is a large, nucleated erythrocyte with delayed and abnormal nuclear maturation. Pernicious anemia is a type of megaloblastic anemia associated with vitamin B2 deficiency. Pathophysiology and Etiology

Vitamin B12 is necessary for normal deoxyribonucleic acid synthesis in maturing RBCs. Pernicious anemia demonstrates familial incidence related to autoimmune gastric mucosal atrophy. Normal gastric mucosa secretes a substance called intrinsic factor, necessary for absorption of vitamin B12 in ileum. If a defect exists in gastric mucosa, or after gastrectomy or small bowel disease, intrinsic factor may not be secreted and orally ingested B12 not absorbed. Some drugs interfere with B12 absorption, notably ascorbic acid, cholestyramine, colchicine, neomycin, cimetidine, and hormonal contraceptives. Primarily a disorder of older people.

Clinical Manifestations

Of anemia pallor, fatigue, dyspnea on exertion, palpitations. May be angina pectoris and heart failure in the elderly or those predisposed to heart disease. Of underlying GI dysfunction sore mouth, glossitis, anorexia, nausea, vomiting, loss of weight, indigestion, epigastric discomfort, recurring diarrhea or constipation. Of neuropathy (occurs in high percentage of untreated patients) paresthesia that involves hands and feet, gait disturbance, bladder and bowel dysfunction, psychiatric symptoms caused by cerebral dysfunction.

Diagnostic Evaluation

CBC and blood smear decreased hemoglobin and hematocrit; marked variation in size and shape of RBCs with a variable number of unusually large cells Folic acid (normal) and B12 levels (decreased). Gastric analysis volume and acidity of gastric juice diminished.
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Schilling test for absorption of vitamin B12 uses small amount of radioactive B12 orally and 24-hour urine collection to measure uptake decreased.

Management Parenteral replacement with hydroxocobalamin or cyanocobalamin (B12) is necessary by I.M. injection from health care provider, generally every month. Complications Neurologic: paresthesia, gait disturbances, bowel and bladder dysfunction, and cerebral dysfunction may be persistent. Nursing Assessment

Assess for pallor, tachycardia, dyspnea on exertion, exercise intolerance to determine patient's response to anemia. Assess for paresthesia, gait disturbances, changes in bladder or bowel function, altered thought processes indicating neurologic involvement. Obtain history of gastric surgery or GI disease.

Nursing Diagnoses

Disturbed Thought Processes related to neurologic dysfunction in absence of vitamin B12 Impaired Sensory Perception (kinesthetic) related to neurologic dysfunction in absence of vitamin B12

Nursing Interventions Improving Thought Processes


Administer parenteral vitamin B12 as prescribed. Provide patient with quiet, supportive environment; reorient to time, place, and person if needed; give instructions and information in short, simple sentences and reinforce frequently.

Minimizing the Effects of Paresthesia


Assess extent and severity of paresthesia, imbalance, or other sensory alterations. Refer patient for physical therapy and occupational therapy as appropriate. Provide safe, uncluttered environment; make sure personal belongings are within reach; provide assistance with activities as needed.

Patient Education and Health Maintenance


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Advise patient that monthly vitamin B12 administration should be continued for life. Instruct patient to see health care provider approximately every 6 months for hematologic studies and GI evaluation; may develop hematologic or neurologic relapse if therapy inadequate.

NURSING ALERT Patients with pernicious anemia have higher incidence of gastric cancer and thyroid dysfunction; periodic stool examinations for occult blood, gastric cytology, and thyroid function tests are done. Evaluation: Expected Outcomes

Oriented, cooperative, and follows instructions Carries out activities without injury

MEGALOBLASTIC ANEMIA: FOLIC ACID DEFICIENCY Chronic megaloblastic anemia caused by folic acid (folate) deficiency. Pathophysiology and Etiology

Dietary deficiency, malnutrition, marginal diets, excessive cooking of foods; commonly associated with alcoholism. Impaired absorption in jejunum (eg, with small bowel disease). Increased requirements (eg, with chronic hemolytic anemia, exfoliative dermatitis, pregnancy). Impaired utilization from folic acid antagonists (methotrexate) and other drugs (phenytoin, broad spectrum antibiotics, sulfamethoxazole, alcohol, hormonal contraceptives).

Clinical Manifestations

Of anemia: fatigue, weakness, pallor, dizziness, headache, tachycardia. Of folic acid deficiency: sore tongue, cracked lips.

Diagnostic Evaluation

Vitamin B12 and folic acid level folic acid will be decreased. CBC will show decreased RBC, hemoglobin, and hematocrit with increased mean corpuscular volume and mean corpuscular hemoglobin concentration.
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Management Oral folic acid replacement on daily basis. Complications Folic acid deficiency has been implicated in the etiology of congenitally acquired neural tube defects. Nursing Assessment

Obtain nutritional history. Monitor level of dyspnea, tachycardia, and development of chest pain or shortness of breath for worsening of condition.

Nursing Diagnosis

Imbalanced Nutrition: Less Than Body Requirements related to inadequate intake of folic acid

Nursing Interventions Improving Folic Acid Intake


Assess diet for inclusion of foods rich in folic acid: beef liver, peanut butter, red beans, oatmeal, broccoli, asparagus. Arrange nutritionist referral as appropriate. Assist alcoholic patient to obtain counseling and additional medical care as needed.

Community and Home Care Considerations


Encourage pregnant patient to maintain prenatal care and to take folic acid supplement. Provide alcoholic patient with information about treatment programs and Alcoholics Anonymous meetings in the community.

Patient Education and Health Maintenance


Teach patient to select balanced diet that includes green vegetables (asparagus, broccoli, spinach), yeast, liver and other organ meats, some fresh fruits; avoid overcooking vegetables. Encourage patient to follow up periodically to monitor CBC.

Evaluation: Expected Outcomes


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Eats appropriate and nutritious diet; takes folic acid supplements as prescribed

APLASTIC ANEMIA Aplastic anemia is a disorder characterized by bone marrow hypoplasia or aplasia resulting in pancytopenia (insufficient numbers of RBCs, WBCs, and platelets). Pathophysiology and Etiology Destruction of hematopoietic stem cells is thought to be through an immune-mediated mechanism.

May be idiopathic or caused by exposure to chemical toxins; ionizing radiation; viral infections, particularly hepatitis; certain drugs (eg, chloramphenicol). May be congenital (Fanconi's anemia). Clinical course is variable and dependent on degree of bone marrow failure; severe aplastic anemia is almost always fatal if untreated.

Clinical Manifestations

From anemia: pallor, weakness, fatigue, exertional dyspnea, palpitations. From infections associated with neutropenia: fever, headache, malaise; adventitious breath sounds; abdominal pain, diarrhea; erythema, pain, exudate at wounds or sites of invasive procedures. From thrombocytopenia: bleeding from gums, nose, GI or GU tracts; purpura, petechiae, ecchymoses.

Diagnostic Evaluation

CBC and peripheral blood smear show decreased RBC, WBC, platelets (pancytopenia). Bone marrow aspiration and biopsy: bone marrow is hypocellular or empty with greatly reduced or absent hematopoiesis.

Management

Removal of causative agent or toxin. Allogeneic bone marrow transplantation (BMT) treatment of choice for patient with severe aplastic anemia. This treatment option provides longterm survival for 75% to 90% of patients, depending on the age of the patient, history of prior blood transfusions, and source of marrow. Immunosuppressive treatment with corticosteroids, cyclosporine, cyclophosphamide, antithymocyte globulin or antilymphocyte globulin as single treatments or in combinations. This treatment option provides long-term survival for 70% to 80% of patients. Androgens (oxymetholone or testosterone enanthate) may stimulate bone marrow regeneration; significant toxicity encountered. They may be used when other treatments have failed. Supportive treatment includes platelet and RBC transfusions, antibiotics, and antifungals.
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Complications

Untreated severe aplastic anemia is almost always fatal, generally because of overwhelming infection. Even with treatment, morbidity and mortality caused by infections and bleeding are high. Late complications, even after successful treatment, include clonal hematologic diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplasia, and acute myelogenous leukemia.

Nursing Assessment

Obtain thorough history that includes medications, past medical history, occupation, hobbies. Monitor for signs of bleeding and infection.

Nursing Diagnoses

Risk for Infection related to granulocytopenia secondary to bone marrow aplasia Risk for Injury related to bleeding

Nursing Interventions Minimizing Risk of Infection


Care for patient in protective environment while hospitalized private room with strict hand washing and avoidance of any contaminants (see Patient Education Guidelines). Encourage good personal hygiene, including daily shower or bath with mild soap, mouth care, and perirectal care after using the toilet. Monitor vital signs, including temperature, frequently; notify health care provider of oral temperature of 101 F (38.3 C) or higher. Minimize invasive procedures or possible trauma to skin or mucous membranes. Obtain cultures of suspected infected sites or body fluids.

Minimizing Risk of Bleeding


Use only soft toothbrush or toothette for mouth care and electric razor for shaving; keep nails short by filing. Avoid I.M. injections and other invasive procedures. Prevent constipation with stool softeners as prescribed. Restrict activity based on platelet count and active bleeding. Monitor pad count for menstruating patient; avoid use of vaginal tampons. Control bleeding by applying pressure to site, using ice packs and prescribed topical hemostatic agents.
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Administer blood product replacement as ordered; monitor for allergic reaction, anaphylaxis, and volume overload.

Patient Education and Health Maintenance

Teach patient how to minimize risk of infection (see Patient Education Guidelines,): o Wash hands after contact with possible source of infection. o Immediately cleanse any abrasion or wound of mucous membranes or skin. o Monitor temperature and report fever or other sign of infection immediately. o Avoid crowds and people with illnesses. o Avoid raw or undercooked foods. o Use condoms and other safer sex practices. Teach patient how to minimize risk of bleeding (see Patient Education Guidelines): o Avoid falls or other injury. o Use electric razor rather than plain razor. o Use nail clippers or file rather than scissors. o Avoid blowing nose. o Use soft toothbrush or toothette for mouth care. o Use water-soluble lubricants as needed during sexual activity. Advise patient to avoid exposure to potential bone marrow toxins: solvents, sprays, paints, pesticides. Teach patient to take only prescribed medications; avoid aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs), which may interfere with platelet function. As some vitamins and herbs may also affect platelet function, instruct patient to check with physician before using any supplements. Resources for patient and family include the Aplastic Anemia Foundation of America, http://www.aamds.org.

PATIENT EDUCATION GUIDELINES Preventing, Recognizing, and Treating Infection What kinds of infections am I at risk for? Because some of your white blood cells (called neutrophils) are not present in their normal numbers, you are at risk for infections. Neutrophils attack bacteria and other organisms that cause infections. You are at risk for infections from bacteria (for example, wound infections, urinary tract infections, pneumonia) and some viral infections (for example, cold sores) and fungal infections (for example, thrush, fungal pneumonia). How do I know when I have an infection? With low numbers of neutrophils, some of the normal signs of infection, such as pus, may not be present. If you have a fever greater than 100 F (37.8 C), you should contact your health care provider immediately. Other signs of infection include chills and sweating, redness near a wound or a catheter, white patches in your mouth, or shortness of breath. How can I protect myself from infection?
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The most important thing you can do is to wash your hands thoroughly after using the bathroom, or after any other dirty activity (such as taking out the trash). You should keep your mouth clean by brushing your teeth with a soft toothbrush after every meal or snack, and by rinsing with a mild mouth rinse. You should shower daily, using a mild soap. Can I have visitors? Can I go out? It is important to ask friends and family not to visit if they are not feeling well. You can certainly go out (unless your physician or nurse gives you different instructions), but you should avoid crowded, poorly ventilated places. You may be taught how to wear a special mask when you are outside. Do I need to get rid of my pet? If you have pets and gardens, you can still enjoy them. If possible, find someone else who will clean your cat's litter box or scoop your dog's waste. Wear gloves when gardening and be careful not to get cuts and abrasions. Should I stop having sex? With your low neutrophil count, you are also at higher risk for sexually transmitted diseases. However, this doesn't mean you have to stop having sex! It is important to use a condom and make sure your sexual practice is safe. Depending on your general health, your physician or nurse may recommend that you avoid sexual intercourse. If I get an infection, how will it be treated? A new infection typically requires hospital admission to diagnose it and to begin treatment. You may be given many tests to find out exactly what infection you have. Blood will be drawn and examined for infection, X-rays and computed tomography scans may be done, and you may need a lumbar puncture to look for an infection in your spinal fluid. Before the results of all the tests are available, your physician will begin treatment with I.V. antibiotics. If your infection does not get better (for example, if you still have a fever in a couple of days), your antibiotics may be changed or other medications may be added. When your infection begins to improve, it is usually possible to go home and continue taking I.V. or oral antibiotics at home. Evaluation: Expected Outcomes

Remains afebrile with no signs or symptoms of infection Episodes of bleeding rapidly controlled

MYELOPROLIFERATIVE DISORDERS Myeloproliferative disorders are disorders of the bone marrow that result from abnormal proliferation of cells from the myeloid line of the hematopoietic system. They include polycythemia vera, acute lymphocytic and acute myelogenous leukemia, and chronic myelogenous leukemia. POLYCYTHEMIA VERA Polycythemia vera is a chronic myeloproliferative disorder that involves all bone marrow elements, resulting in an increase in RBC mass and hemoglobin. PATIENT EDUCATION GUIDELINES Preventing, Recognizing, and Treating Bleeding Why am I at risk for bleeding?
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You could be at risk for bleeding because you do not have enough platelets (cells that help make clots) because your platelets are not working properly or because you do not have enough clotting factors in your blood (substances that help make clots). Some people need treatment with drugs, such as heparin and coumadin, to prevent dangerous clots in the legs and lungs. These drugs increase the risk of bleeding. How will I know if I have bleeding? You might have visible bleeding, such as a nosebleed or bleeding from your gums. You might have very heavy bleeding with your menses (period), which might go on longer than usual. You might notice blood in your stool or black stools (black stools can also occur if you take iron). You might see blood streaks or black flecks in your emesis (vomit). The color of your urine might be pink or red, or you might see red streaks in it. You might have small bruises appear on your skin or redness in the white part of your eyes. If you have bleeding internally you might have symptoms, such as pain, swelling, shortness of breath (bleeding in the lungs), or confusion or stroke symptoms (bleeding in the brain). What should I do to stop something from bleeding? If your nose is bleeding, you can apply pressure by squeezing the bridge of the nose. If you have a wound that is bleeding, apply pressure to the site with a clean or sterile pad. Ice packs can also help; apply ice for 20 minutes at a time, then remove and reapply every hour or two if needed. If you have bleeding in the mouth, biting on a tea bag may also help. If bleeding does not stop quickly or seems to be a lot, you should contact your health care provider or go to an emergency department. You should also seek help right away if you are coughing up blood or vomiting blood, if you feel weak or confused, or if you are worried that it is serious. What can I do to prevent bleeding? There are lots of things you can do to decrease the risk of bleeding. Avoid activities where you have a high risk of injury, eg, contact sports. Use a gentle toothbrush (you can floss gently if it does not cause bleeding). Avoid blowing your nose. Use an electric razor instead of a regular razor. Keep your fingernails and toenails short and smooth using a nail clipper and file rather than scissors. Make sure you do not get constipated straining may cause bleeding. Do not take aspirin, ibuprofen, or naproxen. Acetaminophen (Tylenol) is a safe alternative to these types of medications. Other medications and several vitamins and herbs can increase your risk of bleeding. Do not take over-the-counter medications or herbal and nutritional supplements and vitamins until you have checked with your provider. Take medicine to suppress your menses (period) as ordered. Use water-soluble lubricants during sexual activity to decrease the risk of abrasions. What else should I do? Talk with your health care provider and nurse about your activities. If you are at high risk for bleeding, you may need to restrict or change your exercise and activities. You may need to change sexual practices. Keep your appointments with your health care providers, and take medications as prescribed. If you visit another doctor, dentist, or need surgery, be sure to tell health care providers about your risk for bleeding. Wear a MedicAlert bracelet if you will be at risk for bleeding for some time. Pathophysiology and Etiology

Hyperplasia of all bone marrow elements results in: o Overproduction of all three blood cell lines, most prominently RBCs. o Increased red cell mass. o Increased blood volume and viscosity. o Decreased marrow iron reserve. o Splenomegaly.
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Increased mass of blood cells increases viscosity and leads to engorgement of blood vessels and possible thrombosis. Underlying cause is unknown. Usually occurs in middle and later years.

Clinical Manifestations Result from increased blood volume and viscosity.


Reddish purple skin and mucosa, pruritus (especially after bathing). Splenomegaly, hepatomegaly. Epigastric discomfort, abdominal discomfort. Painful fingers and toes from arterial and venous insufficiency, paresthesia. Headache, fullness in head, dizziness, visual abnormalities, altered mentation from disturbed cerebral circulation. Weakness, fatigue, night sweats, bleeding tendency. Hyperuricemia from increased formation and destruction of erythrocytes and leukocytes and increased metabolism of nucleic acids. Itching related to histamine release from basophils.

Diagnostic Evaluation

CBC elevated RBC and hemoglobin and hematocrit (> 60%); elevated platelets. Bone marrow aspirate and biopsy hyperplasia. Elevated uric acid.

Management

Of hyperviscosity: phlebotomy (withdrawal of blood) at intervals determined by CBC results to reduce RBC mass; generally, 250 to 500 mL removed at a time. Of marrow hyperplasia: myelosuppressive therapy, generally using hydroxyurea (Hydrea) or I.V. radioactive phosphorus; biologic response modifier, ie, alpha-interferon. Of hyperuricemia: allopurinol (Zyloprim). Of pruritus: antihistamines (cimetidine [Tagamet] or cyproheptadine [Periactin]); low-dose aspirin; certain antidepressants (doxepin [Sinequan], paroxetine [Paxil]); phototherapy; cholestyramine (Questran).

Complications

Thromboembolic events caused by hyperviscosity, including deep vein thrombophlebitis, myocardial and cerebral infarction, transient ischemic attacks, pulmonary embolism, retinal vein thrombosis, and thrombotic occlusion of the splenic, hepatic, portal, and mesenteric veins.
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Spontaneous hemorrhage caused by venous and capillary distention and abnormal platelet function. Gout caused by hyperuricemia. Heart failure caused by increased blood volume and hypertension. Myelofibrosis or acute myeloid leukemia may be terminal complications.

Nursing Assessment

Obtain history of symptoms, including changes in skin, epigastric discomfort, bleeding tendencies, circulatory problems, or painful, swollen joints. Monitor for signs of bleeding or thromboembolism. Monitor for hypertension and signs and symptoms of heart failure, including shortness of breath, distended neck veins.

Nursing Diagnosis

Ineffective Tissue Perfusion (multiple organs) related to hyperviscosity of blood

Nursing Interventions Preventing Thromboembolic Complications


Encourage or assist with ambulation. Assess for early signs of thromboembolic complicationsswelling of limb, increased warmth, pain. Monitor CBC and assist with phlebotomy as ordered.

Patient Education and Health Maintenance


Educate patient about risk of thrombosis; encourage patient to maintain normal activity patterns and avoid long periods of bed rest. Advise patient to avoid taking hot showers or baths because rapid skin cooling worsens pruritus; use skin emollients; take antihistamines as prescribed; may find starch baths helpful. Instruct patient to take only prescribed medications. Encourage patient to report at prescribed intervals for follow-up blood (hematocrit) studies and phlebotomies. Instruct patient in technique of subcutaneous injection for alpha-interferon.

Evaluation: Expected Outcomes

Hematocrit less than 45% in men and less than 42% in women; no signs or symptoms of thromboembolism, heart failure, or bleeding
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ACUTE LYMPHOCYTIC AND ACUTE MYELOGENOUS LEUKEMIA See Nursing Care Plan 26-1 Leukemias are malignant disorders of the blood and bone marrow that result in an accumulation of dysfunctional, immature cells that are caused by loss of regulation of cell division. They are classified as acute or chronic based on the development rate of symptoms, and further classified by the predominant cell type. Acute leukemias affect immature cells and are characterized by rapid progression of symptoms. When lymphocytes are the predominant malignant cell, the disorder is acute lymphocytic leukemia (ALL); when monocytes or granulocytes are predominant, it is acute myelogenous leukemia (AML), sometimes called acute nonlymphocytic leukemia. Pathophysiology and Etiology

The development of leukemia has been associated with: o Exposure to ionizing radiation. o Exposure to certain chemicals and toxins (eg, benzene, alkylating agents). o Human T-cell leukemialymphoma virus (HTLV-1 and HTLV-2) in certain areas of the world, including the Caribbean and southern Japan. o Familial susceptibility. o Genetic disorders (eg, Down syndrome, Fanconi's anemia). Approximately half of new leukemias are acute. Approximately 85% of acute leukemias in adults are AML. ALL is most common in children, with peak incidence between ages 2 and 9. Childhood ALL is usually cured with chemotherapy alone (> 75%), whereas only 30% to 40% of adults with ALL are cured. AML is a disease of older people, with a median age at diagnosis of 67. Even in the young-old (patients who are younger than age 60), AML is difficult to treat, with a median survival of 5 to 6 months, despite intensive therapy.

Clinical Manifestations

Common symptoms include pallor, fatigue, weakness, fever, weight loss, abnormal bleeding and bruising, lymphadenopathy (in ALL), and recurrent infections (in ALL). Other presenting symptoms may include bone and joint pain, headache, splenomegaly, hepatomegaly, neurologic dysfunction.

Diagnostic Evaluation

CBC and blood smear peripheral WBC count varies widely from 1,000 to 100,000/mm3 and may include significant numbers of abnormal immature (blast) cells; anemia may be profound; platelet count may be abnormal and coagulopathies may exist. Bone marrow aspiration and biopsy cells also studied for chromosomal abnormalities (cytogenetics) and immunologic markers to classify type of leukemia further. Lymph node biopsy to detect spread. Lumbar puncture and examination of cerebrospinal fluid for leukemic cells (especially in ALL).
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Management

To eradicate leukemic cells and allow restoration of normal hematopoiesis. o High-dose chemotherapy given as an induction course to obtain a remission (disappearance of abnormal cells in bone marrow and blood) and then in cycles as consolidation or maintenance therapy to prevent recurrence of disease (see Table 26-3). TABLE 26-3 Common Chemotherapeutic Drugs Used in Acute Leukemias DRUG MAJOR ADVERSE EFFECTS CLASSIFICATION PRIMARY USE Cytarabine (ARA-C, Bone marrow suppression, nausea and vomiting, Antimetabolite Induction and consolidation therapy Cytosar-U) pulmonary toxicity, mucositis, lethargy, cerebellar for AML toxicity, dermatitis, keratoconjunctivitis Daunorubicin Bone marrow suppression, nausea and vomiting, Antibiotic Induction and consolidation therapy (Cerubidine) alopecia, cardiotoxicity, vesicant for AML Doxorubicin Leukopenia, nausea and vomiting, alopecia, Antibiotic Induction and consolidation therapy (Adriamycin PFS) cardiotoxicity, photosensitivity, vesicant for AML L-asparaginase Liver dysfunction, nausea and vomiting, Miscellaneous: Induction therapy for ALL (Elspar) hypersensitivity reaction, depression, lethargy enzyme 6-Mercaptopurine (6- Mild bone marrow suppression, GI disturbances, Antimetabolite Maintenance therapy for ALL MP, Purinethol) hepatotoxicity Methotrexate Bone marrow suppression, stomatitis, nausea, Antimetabolite Intrathecal central nervous system (Rheumatex, Trexall) diarrhea, hepatotoxicity, neurotoxicity with treatment and prophylaxis for ALL; intrathecal doses maintenance therapy for ALL Prednisone (Orasone) Appetite stimulation, mood alteration, Cushing's Corticosteroid Induction therapy for ALL syndrome, hypertension, diabetes, peptic ulcer Vincristine (Oncovin, Neurotoxicity, alopecia, vesicant Plant alkaloid Induction therapy for ALL Vincasar) ALL: acute lymphocytic leukemia; AML: acute myelogenous leukemia. Leukapheresis (or exchange transfusion in infants) may be used when abnormally high numbers of white cells are present to reduce the risk of leukostasis and tumor burden before chemotherapy. o Radiation, particularly of central nervous system (CNS) in ALL. o Autologous or allogeneic bone marrow or stem cell transplantation. Supportive care and symptom management.
o

NURSING CARE PLAN 26-1 Care of the Patient with Acute Leukemia
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You are assigned Charles Wintry, a 48-year-old with acute myelogenous leukemia (AML) who has been admitted with neutropenia and thrombocytopenia following chemotherapy. From your assessment and your knowledge of AML, you develop your care plan. Subjective data: Charles tells you he has felt very tired during the past week and has stopped working at his part-time store manager job. Since yesterday evening, he has had a low-grade fever and pain in his chest with inspiration. He has also noticed new bruises on his lower legs and abdomen. This morning he had a nosebleed. Objective data: Vital signs are temperature 101 F (38.3 C), pulse 104, blood pressure 145/90, respirations 32. On examination, you notice extensive petechiae; dried blood around the nares and gingival bleeding; warm, dry skin; and crackles heard in the bases of both lungs. Complete blood count reveals 30,000 platelets, 1,200 white blood cells with 450 neutrophils, and hematocrit of 32%. NURSING DIAGNOSIS Risk for Infection related to granulocytopenia secondary to leukemia or its treatment with chemotherapy or radiation GOAL/EXPECTED OUTCOME Risk of infection will be minimized. Nursing Intervention Rationale Evaluation: Outcome Place patient in private room with hand-washing precautions Meticulous hand washing is the single most Staff and visitors comply with strictly enforced. important method of preventing transmission of hand-washing precautions. endogenous and exogenous nosocomial infections. Avoid exposure to all sources of stagnant water (eg, flower Stagnant water and soil are good media for No visible sources of bacterial vases, denture cups, water pitchers, humidifiers) and plants. anaerobic bacterial growth. growth found in room. Encourage or assist with personal hygienemouth care, Skin care and good oral hygiene may help prevent Skin remains clean and perirectal care, daily shower or bath with mild soap. Inspect skin skin, oral, respiratory, and GI infections. Signs of lubricated. Oropharynx has no and mucous membranes daily for possible signs of infection. infection may be minimal in neutropenic patient. lesions, plaques, or erythema. Monitor vital signs q4h. Obtain baseline pulse oximeter reading Infections may progress rapidly in Vital signs stable with no (SaO2). immunocompromised patient. changes to report to health care provider. Assess respiratory function q4h while symptoms present, Neutropenic patient may have significant bacterial Ambulates and uses incentive otherwise q8h. Encourage ambulation, deep breathing, and or fungal pneumonia with minimal changes on chest spirometer q4h. coughing. X-ray or physical examination. Assess for changes in mental status at least q8h, including Mental status changes are commonly first subtle Mental status remains stable. restlessness, irritability, confusion, headache, or changes in level signs of sepsis. Patient alert and oriented. of consciousness. Avoid invasive procedures if possible, eg, urinary Risk of infection from invasive procedure is high. No invasive procedures catheterization. Use strict aseptic technique if procedure initiated. Injections given I.V. unavoidable. Prevent rectal trauma by avoiding rectal temperatures, enemas, Perianal area is high-risk site for infection, including Perianal area remains clean or suppositories. Use sitz bath and barrier cream for patient with rectal abscesses. and intact.
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diarrhea and hemorrhoids. Use stool softeners as needed to prevent constipation. Obtain cultures of suspected infected sites or body fluids.

Pus may not be present with neutropenia. Cultures may reveal bacterial, fungal, or viral pathogens. Report: 1) fever = 101 F (38.3 C) once or 100.4 F (38 C) two Fever may be only response to infection in times; 2) significant change in vital signs, particularly neutropenic patient. Patient is at risk for sepsis due hypotension, tachycardia, or tachypnea; 3) chills or rigors; 4) to lack of normal immunologic response. mental status changes. Teach patient measures to prevent infection: avoid crowds; avoid Potential for infection remains after discharge. raw or undercooked food; use condoms. NURSING DIAGNOSIS Risk for Injury related to bleeding secondary to thrombocytopenia, disseminated intravascular coagulation or leukostasis associated with leukemia GOAL/EXPECTED OUTCOME Risk of bleeding will be minimized. Nursing Intervention Assess for signs of bleeding at least q8h. Provide soft toothbrush or toothettes and mild mouthwash for mouth care. Use only an electric razor for shaving. Keep fingernails and toenails short and smooth. Lubricate skin with mild lotion. Avoid I.M. injections, invasive procedures, rectal procedures. Use stool softeners to prevent constipation. Restrict activity based on assessment of platelet count and presence of active bleeding.

Sputum sent for culture. Fever of 101.2 F (38.4 C) reported.

Patient states preventative measures.

Menstrual suppression may be necessary for females. Monitor pad count/amount. Avoid use of vaginal tampons. Control bleeding by applying pressure to site, ice packs, and prescribed topical hemostatic agents, such as microfibrillar

Rationale Evaluation: Outcome Overt and covert spontaneous bleeding is possible at No further bleeding noted. platelet counts < 50,000/mm3. Minimize damage to mucous membranes. Uses soft toothbrush after meals for gentle cleansing. Minimize skin excoriation. Wife brings electric razor from home. Minimize risk of bleeding. Invasive procedures minimized. Injections given I.V. Injury to muscles or spontaneous bleeding may be Ambulates within room with minimized by restricting activity at lower platelet assistance until bleeding counts. controlled and platelet count stabilized. Menorrhagia may be severe in thrombocytopenic Menstrual bleeding not patient. exceeding four to five pads per day. Topical interventions generally adequate to control No further bleeding episodes. most bleeding episodes.
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collagen hemostat (Avitene) or thrombin (Thrombinar). Administer blood product replacement as ordered. Monitor for signs and symptoms of allergic reactions, anaphylaxis, and volume overload.

Teach patient to avoid activities likely to cause injury (eg, contact sports) and other methods to prevent bleeding. NURSING DIAGNOSIS Pain related to tumor growth, infection, or adverse effects of chemotherapy Nursing Intervention Rationale Evaluation: Outcome Assess at least q4h for presence, location, intensity, and Pain is potentially distressing symptom. Pain may be Patient rates pain between 1 characteristics of pain. symptom of infection. and 3 on scale of 0-10 after first day. Administer analgesics as ordered to control pain. Administer on Analgesics on regular schedule at appropriate doses Oral analgesia (codeine 30 mg regular schedule rather than as needed. Avoid aspirin and should be used to control pain. Aspirin and NSAIDs q4h) well tolerated. nonsteroidal anti-inflammatory drugs (NSAIDs) in interfere with platelet function. I.M. and S.C. thrombocytopenic patients. If oral analgesics in optimal doses injections should be avoided in thrombocytopenia. are not effective or not tolerated, consider I.V. route.

Platelet transfusions may be necessary when platelet Platelet products given q2-3d count < 20,000/mm3 or with active bleeding. Other until thrombocytopenia blood products (red blood cells, fresh frozen plasma, resolved. cryoprecipitate) may also be required. Risk for bleeding remains after discharge. Patient states preventative measures.

Teach and use nonpharmacologic measures, such as the use of Nonpharmacologic measures may be useful music, relaxation breathing, progressive muscle relaxation, adjunctive treatments for patients with pain. distraction and imagery to help manage pain. NURSING DIAGNOSIS Powerlessness related to diagnosis and perceived lack of support/resources GOAL/EXPECTED OUTCOME Patient and family will be empowered to seek appropriate help. Nursing Intervention Rationale Encourage verbalization of feelings regarding diagnosis, Nurse-patient relationship allows appropriate treatment plan, and anticipated course of illness. discussion of concerns. Refer as needed to social worker, psychiatric liaison nurse, Further assistance and support may be needed. psychologist. Share information regarding national and local resources.

Heat packs to right chest area provide additional relief.

Evaluation: Outcome Verbalizes feelings through discussion, crying. Referred to social worker for assistance with financial concerns. National and local organizations may be significant Attends American Cancer
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sources of support for patients and families.

Society support group after discharge.

Complications

Leukostasis: in setting of high numbers (greater than 50,000/mm3) of circulating leukemic cells (blasts), blood vessel walls are infiltrated and weakened, with high risk of rupture and bleeding, including intracranial hemorrhage. Disseminated intravascular coagulation (DIC). Tumor lysis syndrome: rapid destruction of large numbers of malignant cells leads to alterations in electrolytes (hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia). May lead to renal failure and other complications. Infection, bleeding, organ damage. DRUG ALERT
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Allopurinol is commonly used as part of a regimen to prevent tumor lysis syndrome. In rare cases, it causes severe, even lethal, skin reactions (toxic epidermolysis syndrome). Allopurinol should be discontinued for any patient who develops a new skin rash. Nursing Assessment

Take nursing history, focusing on weight loss, fever, frequency of infections, progressively increasing fatigability, shortness of breath, palpitations, visual changes (retinal bleeding). Ask about difficulty in swallowing, coughing, rectal pain. Examine patient for enlarged lymph nodes, hepatosplenomegaly, evidence of bleeding, abnormal breath sounds, skin lesions. Look for evidence of infection: mouth, tongue, and throat for reddened areas or white patches. Examine skin for breakdown, which is a potential source of infection.

Nursing Diagnoses

Risk for Infection related to granulocytopenia of disease and treatment Risk for Injury related to bleeding secondary to bone marrow failure and thrombocytopenia

Nursing Interventions Preventing Infection


Especially monitor for pneumonia, pharyngitis, esophagitis, perianal cellulitis, urinary tract infection, and cellulitis, which are common in leukemia and which carry significant morbidity and mortality. Monitor for fever, flushed appearance, chills, tachycardia; appearance of white patches in mouth; redness, swelling, heat or pain of eyes, ears, throat, skin, joints, abdomen, rectal and perineal areas; cough, changes in sputum; skin rash. Check results of granulocyte counts. Concentrations less than 500/mm3 put the patient at serious risk for infection. Avoid invasive procedures and trauma to skin or mucous membrane to prevent entry of microorganisms. Use the following rectal precautions to prevent infection: o Avoid diarrhea and constipation, which can irritate the rectal mucosa. o Avoid use of rectal thermometers. o Keep perianal area clean. Care for patient in private room with strict hand-washing practice. Patients with prolonged neutropenia may benefit from HEPA filtration. Encourage and assist patient with personal hygiene, bathing, and oral care. Obtain cultures and administer antimicrobials promptly as directed.

Preventing and Managing Bleeding


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Watch for signs of minor bleeding, such as petechiae, ecchymosis, conjunctival hemorrhage, epistaxis, bleeding gums, bleeding at puncture sites, vaginal spotting, heavy menses. Be alert for signs of serious bleeding, such as headache with change in responsiveness, blurred vision, hemoptysis, hematemesis, melena, hypotension, tachycardia, dizziness. Test all urine, stool, emesis for gross and occult blood. Monitor platelet counts daily. Administer blood components as directed. Keep patient on bed rest during bleeding episodes.

Patient Education and Health Maintenance


Teach infection precautions (see Patient Education Guidelines). Teach signs and symptoms of infection and advise whom to notify. Encourage adequate nutrition to prevent emaciation from chemotherapy. Teach avoidance of constipation with increased fluid and fiber, and good perianal care. Teach bleeding precautions (see Patient Education Guidelines). Encourage regular dental visits to detect and treat dental infections and disease. Provide patient and family with information about resources in the community, such as the Leukemia Society of America and the American Cancer Society (see Box 26-1).

Evaluation: Expected Outcomes


Afebrile, without signs of infection No signs of bleeding

CHRONIC MYELOGENOUS LEUKEMIA Chronic myelogenous leukemia (CML) (ie, involving more mature cells than acute leukemia) is characterized by proliferation of myeloid cell lines, including granulocytes, monocytes, platelets, and occasionally RBCs. Pathophysiology and Etiology

Specific etiology unknown, associated with exposure to ionizing radiation and family history of leukemia. Results from malignant transformation of pluripotent hematopoietic stem cell. First cancer associated with chromosomal abnormality (the Philadelphia [Ph] chromosome), present in more than 90% of patients. Accounts for 25% of adult leukemias and less than 5% of childhood leukemias. Generally presents between ages 25 and 60 with peak incidence in the mid-40s.
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With progression of illness, enters terminal phase, resembling an acute leukemia that consists of accelerated phase or blast crisis.

Clinical Manifestations

Insidious onset, may be discovered during routine physical examination. Common symptoms include fatigue, pallor, activity intolerance, fever, weight loss, night sweats, abdominal fullness (splenomegaly).

Diagnostic Evaluation

CBC and blood smear: large numbers of granulocytes (usually more than 100,000/mm3), platelets may be decreased. Bone marrow aspiration and biopsy: hypercellular, usually demonstrates Philadelphia (Ph1) chromosome.

Management Chronic Phase

The introduction of imatinib (Gleevec) in 2001 changed treatment options for patients with CML, providing a highly effective oral treatment for newly diagnosed patients as well as for patients in chronic or accelerated phases. A protein-tyrosine kinase inhibitor, it works by inhibiting proliferation of abnormal cells and inducing cell death (apoptosis) in abnormal cells. Adverse effects include edema, GI irritation, hematologic toxicity and, rarely, hepatotoxicity. For patients who cannot tolerate imatinib mesylate, alpha interferon frequently eliminates the Ph1 chromosome and blasts. Adverse effects (most commonly fatigue and fevers) may be severe. Other options include allogeneic (related or unrelated donor) BMT. Palliative treatment, controlling symptoms, includes chemotherapy with such agents as busulfan (Myleran) or hydroxyurea (Hydrea); irradiation; splenectomy.

Accelerated Phase or Blast Crisis


If imatinib is ineffective, high-dose chemotherapy (usually AML regimens) and leukophoresis may be used to attempt to regain chronic phase. Supportive care and palliative care because this phase is usually terminal.

Complications

Leukostasis. Infection, bleeding, organ damage.


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Untreated, CML is a terminal disease with unpredictable survival, on average 3 years.

Nursing Assessment

Obtain health history, focusing on fatigue, weight loss, night sweats, activity intolerance. Assess for signs of bleeding and infection. Evaluate for splenomegaly, hepatomegaly. Assess for weight gain and edema in patients taking imatinib.

Nursing Diagnosis

Fear related to disease progression and death

Nursing Interventions For patient with CML in blast crisis, see Nursing Care Plan 26-1. Allaying Fear

Encourage appropriate verbalization of feelings and concerns. Provide comprehensive patient teaching about disease, using methods and content appropriate to patient's needs. Assist patient in identifying resources and support (eg, family and friends, spiritual support, community or national organizations, support groups). Facilitate use of effective coping mechanisms.

Patient Education and Health Maintenance


Teach patient to take medications as prescribed and monitor for adverse effects. Teach patient method of subcutaneous injection for self-administration of alpha interferon, and teach strategies for managing such adverse effects as fatigue and fevers. Provide patient and family with information about resources in the community, such as the Leukemia and Lymphoma Society and the American Cancer Society (see Box 26-1).

Evaluation: Expected Outcomes

Demonstrates effective coping skills

LYMPHOPROLIFERATIVE DISORDERS
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Lymphoproliferative disorders result from proliferation of cells from the lymphoid line of the hematopoietic system. They include chronic lymphocytic leukemia, Hodgkin's lymphoma, non-Hodgkin's lymphomas, and multiple myeloma. CHRONIC LYMPHOCYTIC LEUKEMIA Chronic lymphocytic leukemia (CLL) (ie, involving more mature cells than acute leukemia) is characterized by proliferation of morphologically normal but functionally inert lymphocytes. Classified according to cell origin, it includes B cell (accounts for 95% of cases), T cell, lymphosarcoma, and prolymphocytic leukemia. The differential diagnosis includes hairy cell leukemia and Waldenstrom's macroglobulinemia. Pathophysiology and Etiology

Specific etiology unknown. Tends to cluster in families, much more common in Western hemisphere. Male hormones may play role. Most common adult leukemia in United States and Europe. Disease of later years (90% over age 50); twice as common in men than in women. Lymphocytes are immuno-incompetent and respond poorly to antigenic stimulation. In late stages, organ damage may occur from direct lymphocytic infiltration of tissue. May be indolent for years, with gradual transformation to more malignant disease.

Clinical Manifestations

Insidious onset, may be discovered during routine physical examination. Early symptoms may include history of frequent skin or respiratory infections, symmetrical lymphadenopathy, mild splenomegaly. Symptoms of more advanced disease include pallor, fatigue, activity intolerance, easy bruising, skin lesions, bone tenderness, abdominal discomfort.

Diagnostic Evaluation

CBC and blood smear: large numbers of lymphocytes (10,000 to 150,000/mm3); may also be anemia, thrombocytopenia, hypogammaglobulinemia. Bone marrow aspirate and biopsy: lymphocytic infiltration of bone marrow. Lymph node biopsy to detect spread.

Management Symptom Control and Treatments


Patient with newly diagnosed CLL is generally observed and followed closely until symptoms develop. Lymphocyte proliferation can be suppressed with chlorambucil (Leukeran), cyclophosphamide (Cytoxan), and prednisone (Orasone). B cell CLL may be treated with fludarabine (Fludara).
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Monoclonal antibodies such as alemtuzumab (Campath) and rituximab (Rituxan) may be used. Hairy cell leukemia, a distinctive type of B cell leukemia with hairlike projections of cytoplasm from lymphocytes, may be successfully treated with cladribine (Leustatin), pentostatin (Nipent), or alpha interferon. Splenic irradiation or splenectomy for painful splenomegaly or platelet sequestration, hemolytic anemia. Irradiation of painful enlarged lymph nodes. Bone marrow transplant and combinations of alpha interferon and interleukin-2 are also used to treat CLL.

Supportive Care

Transfusion therapy to replace platelets and RBCs. Antibiotics, antivirals, and antifungals as needed to control infections. I.V. immunoglobulins or gamma globulin to treat hypogammaglobulinemia.

Complications

Thrombophlebitis from venous or lymphatic obstruction caused by enlarged lymph nodes. Infection, bleeding. Median survival depends on severity of disease; varies from 2 to 7 years.

Nursing Assessment

Obtain health history, focusing on history of infections, fatigue, bruising and bleeding, swollen lymph nodes. Assess for signs of anemia, bleeding, or infection. Evaluate for splenomegaly, hepatomegaly, lymphadenopathy.

Nursing Diagnoses

Acute pain related to tumor growth, infection, or adverse effects of chemotherapy Activity Intolerance related to anemia and adverse effects of chemotherapy

Nursing Interventions Reducing Pain

Assess patient frequently for pain and administer or teach patient to administer analgesics on regular schedule, as prescribed; monitor for adverse effects.
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Teach patient the use of nonpharmacologic methods, such as the use of music, relaxation breathing, progressive muscle relaxation, distraction, and imagery to help to manage pain.

Improving Activity Tolerance


Encourage frequent rest periods alternating with ambulation and light activity as tolerated. Assist patient with hygiene and physical care as necessary. Encourage balanced diet or nutritional supplements as tolerated. Teach patient to use energy conservation techniques while performing activities of daily living, such as sitting while bathing, minimizing trips up and down stairs, using shoulder bag or push cart to carry articles.

Patient Education and Health Maintenance


Teach patient to minimize risk of infection (see Patient Education Guidelines, page 944). Teach patient use of medications as ordered, and possible adverse effects and their management; also teach patient to avoid aspirin and NSAIDs, which may interfere with platelet function. Provide patient and family with information about resources in the community, such as the Leukemia and Lymphoma Society and the American Cancer Society (see Box 26-1).

Evaluation: Expected Outcomes


States free from pain Performs activities without complaints of fatigue

HODGKIN'S LYMPHOMA Lymphomas are malignant disorders of the reticuloendothelial system that result in an accumulation of dysfunctional, immature lymphoid-derived cells. They are classified according to the predominant cell type and by the degree of malignant cell maturity (eg, well differentiated, poorly differentiated, or undifferentiated). Hodgkin's lymphoma originates in the lymphoid system and involves predominantly lymph nodes. Pathophysiology and Etiology

Etiology is unknown. Characterized by appearance of Reed-Sternberg multinucleated giant cell in tumor. Generally spreads via lymphatic channels, involving lymph nodes, spleen, and ultimately extralymphatic sites. May also spread via bloodstream to such sites as GI tract, bone marrow, skin, upper air passages, and other organs. Incidence demonstrates two peaks, between ages 20 and 40 and after age 60. Risk is increased in males, in individuals with previous EpsteinBarr viral infection, and in individuals with first-degree relative with Hodgkin's lymphoma.
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Clinical Manifestations

Common symptoms include painless enlargement of lymph nodes (generally unilateral), fever, chills, night sweats, weight loss, pruritus. Various symptoms may occur with pulmonary involvement, superior vena cava obstruction, hepatic or bone involvement.

Diagnostic Evaluation Tests are used to determine extent of disease involvement before treatment and followed at regular intervals to assess response to treatment.

CBC determines abnormal cells. Lymph node biopsy determines type of lymphoma. Bilateral bone marrow aspirate and biopsy determine whether bone marrow is involved. Radiographic tests (eg, X-rays, computed tomography [CT] scan, magnetic resonance imaging [MRI]) to detect deep nodal involvement. Gallium-67 scandetects areas of active disease and may be used to determine aggressiveness of disease. Liver function tests, scan to determine hepatic involvement; liver biopsy may be indicated if results abnormal. Lymphangiogram to detect size and location of deep nodes involved, including abdominal nodes, which may not be readily seen via CT scan. Surgical staging (laparotomy with splenectomy, liver biopsy, multiple lymph node biopsies) in selected patients.

Management Choice of treatment depends on extent of disease, histopathologic findings, and prognostic indicators. Hodgkin's lymphoma is more readily cured than other lymphomas, with a 5-year survival of 80%. More than one treatment strategy is available, and combinations of radiation and chemotherapy are commonly used. Hodgkin's lymphoma arising in presence of HIV requires specialized treatment.

Radiation therapy o Treatment of choice for localized disease. o Areas of body where lymph node chains are located can generally tolerate high radiation doses. o Vital organs are protected with lead shielding during radiation treatments. Chemotherapy o Initial treatment commonly with ABVD regimen of doxorubicin (Adriamycin), bleomycin (Blenoxane), vinblastine (Velban), and dacarbazine (DTIC) or MOPP regimen of nitrogen mustard (Mustargen), vincristine (Oncovin), procarbazine (Matulane), and prednisone. o Three or four drugs may be given in intermittent or cyclical courses with periods off treatment to allow recovery from toxicities. Autologous or allogeneic bone marrow or stem cell transplantation

Complications

Adverse effects of radiation or chemotherapy.


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Dependent on location and extent of malignancy, but may include splenomegaly, hepatomegaly, thromboembolic complications, spinal cord compression.

Nursing Assessment

Obtain health history, focusing on fatigue, fever, chills, night sweats, swollen lymph nodes. Evaluate splenomegaly, hepatomegaly, lymphadenopathy.

Nursing Diagnoses

Impaired Tissue Integrity related to high-dose radiation therapy Impaired Oral Mucous Membrane related to high-dose radiation therapy

Nursing Interventions Maintaining Tissue Integrity


Avoid rubbing, powders, deodorants, lotions, or ointments (unless prescribed) or application of heat and cold to treated area. Encourage patient to keep treated area clean and dry, bathing area gently with tepid water and mild soap. Encourage wearing loose-fitting clothes. Advise patient to protect skin from exposure to sun, chlorine, temperature extremes.

Preserving Oral and GI Tract Mucous Membranes


Encourage frequent small meals, using bland and soft diet at mild temperatures. Teach patient to avoid irritants, such as alcohol, tobacco, spices, extreme food temperatures. Administer or teach self-administration of pain medication or antiemetic before eating or drinking, if needed. Encourage mouth care at least twice per day and after meals using gentle flossing, soft toothbrush or toothette, and mild mouth rinse. Assess for ulcers, plaques, or discharge that may be indicative of superimposed infection. For diarrhea, switch to low-residue diet and administer antidiarrheals as ordered.

Patient Education and Health Maintenance


Teach patient about risk of infection (see Patient Education Guidelines). Teach patient how to take medications as ordered, and instruct about possible adverse effects and management.
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Explain to patient that radiation therapy may cause sterility; men should be given opportunity for sperm banking before treatment; women may develop ovarian failure and require hormone replacement therapy. Reassure patient that fatigue will decrease after treatment is completed; encourage frequent naps and rest periods. Provide patient and family with information about resources in the community, such as the Leukemia and Lymphoma Society and the American Cancer Society (see Box 26-1).

Evaluation: Expected Outcomes


Skin intact without erythema or swelling Oral mucosa intact, patient eating

NON-HODGKIN'S LYMPHOMAS Non-Hodgkin's lymphomas are a group of malignancies of lymphoid tissue arising from T or B lymphocytes or their precursors; include both indolent and aggressive forms. Pathophysiology and Etiology

Association with defective or altered immune system; higher incidence in patients receiving immunosuppression for organ transplant, in HIVpositive people, and with some viruses (eg, HTLV-1 and Epstein-Barr). Other risk factors include male gender, white ethnicity, history of Helicobacter gastritis, history of Hodgkin's lymphoma, history of radiation therapy, diet high in meats and fat, exposure to certain pesticides. Arise from malignant transformation of lymphocyte at some stage during development; level of differentiation and type of lymphocyte influences course of illness and prognosis. Incidence rises steadily from age 40.

Clinical Manifestations

Common symptoms include painless enlargement of lymph nodes (generally unilateral), fever, chills, night sweats, weight loss, unexplained pain in chest, abdomen, or bones. Unlike Hodgkin's lymphoma, is more likely to be advanced disease at presentation. Various symptoms may occur with pulmonary involvement, superior vena cava obstruction, hepatic or bone involvement.

Diagnostic Evaluation

Lymph node biopsy to detect type. CBC, bone marrow aspirate and biopsy to detect bone marrow involvement. X-rays, CT scan, positron-emission tomography scan, Gallium scan, and MRI to detect deep nodal involvement. Liver function tests, liver scan to detect liver involvement.
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Lumbar puncture to detect CNS involvement. Surgical staging (laparotomy with splenectomy, liver biopsy, multiple lymph node biopsies).

Management

Radiation therapy generally palliative, not curative. Chemotherapy: various regimens available, including CHOP regimen of cyclophosphamide (Cytoxan), doxorubicin (Adriamycin), vincristine (Oncovin), and prednisone (Orasone) or BACOP regimen of bleomycin (Blenoxane), doxorubicin (Adriamycin), cyclophosphamide (Cytoxan), vincristine (Oncovin), and prednisone. Monoclonal antibody therapy: rituximab (Rituxan), Yttrium-90-labeled ibritumomab tiuxetan (Zevalin), generally given with combination chemotherapy. Autologous or allogeneic bone marrow or stem cell transplantation.

Complications

Complications of radiation therapy and chemotherapy (see pages 141 and 152). Of disease: depends on location and extent of malignancy, but may include splenomegaly, hepatomegaly, thromboembolic complications, spinal cord compression.

Nursing Assessment

Obtain health history, focusing on fatigue, fever, chills, night sweats, swollen lymph nodes, and history of illness or therapy causing immunosuppression. Evaluate splenomegaly, hepatomegaly, lymphadenopathy.

Nursing Diagnosis

Risk for Infection related to altered immune response because of lymphoma and leukopenia caused by chemotherapy or radiation therapy

Nursing Interventions Minimizing Risk of Infection


Care for patient in protected environment with strict hand washing observed. Avoid invasive procedures, such as urinary catheterization, if possible. Assess temperature and vital signs, breath sounds, LOC, and skin and mucous membranes frequently for signs of infection.
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Notify health care provider of fever greater than 101 F (38.3 C) or change in condition. Obtain cultures of suspected infected sites or body fluids.

Patient Education and Health Maintenance


Teach patient infection precautions (see Patient Education Guidelines). Encourage frequent follow-up visits for monitoring of CBC and condition. Provide patient and family with information about resources in the community, such as the Leukemia and Lymphoma Society and the American Cancer Society (see Box 26-1).

Evaluation: Expected Outcomes

Remains afebrile with no signs or symptoms of infection

MULTIPLE MYELOMA Multiple myeloma is a malignant disorder of plasma cells. Pathophysiology and Etiology

Etiology unknown; genetic and environmental factors, such as chronic exposure to low levels of ionizing radiation, may play a part. Characterized by proliferation of neoplastic plasma cells derived from one B lymphocyte (clone) and producing a homogeneous immunoglobulin (M protein or Bence Jones protein) without any apparent antigenic stimulation. Plasma cells produce osteoclast-activating factor leading to extensive bone loss, severe pain, and pathologic fractures. Abnormal immunoglobulin affects renal function, platelet function, resistance to infection, and may cause hyperviscosity of blood. Generally affects older people (median age at diagnosis is 68) and is more common among black men and women.

Clinical Manifestations

Constant, usually severe bone pain caused by bone lesions and pathologic fractures; sites commonly affected include thoracic and lumbar vertebrae, ribs, skull, pelvis, and proximal long bones. Fatigue and weakness related to anemia caused by crowding of marrow by plasma cells. Proteinuria and renal insufficiency. Electrolyte disturbances, including hypercalcemia (bone destruction), hyperuricemia (cell death, renal insufficiency).

Diagnostic Evaluation
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Bone marrow aspiration and biopsy demonstrate increased number and abnormal form of plasma cells. CBC and blood smear changes reflect anemia. Urine and serum analysis for presence and quantity of abnormal immunoglobulin. Skeletal X-rays osteolytic bone lesions.

Management

Chemotherapy, including oral melphalan (Alkeran) or cyclophosphamide (Cytoxan), high-dose corticosteroids alone or in combination with chemotherapy. Thalidomide (Thalomid), an antiangiogenesis agent, with or without chemotherapy or high-dose corticosteroids. Alpha interferon as maintenance therapy. Bortezomib (Velcade), a proteasome inhibitor, for treatment of relapse. Autologous or allogeneic bone marrow or peripheral blood stem cell transplant in selected cases (usually younger than age 50 with no renal failure, few bone lesions, and good organ function). Supportive care options: o Plasmapheresis to treat hyperviscosity or bleeding. o Radiation therapy for bone lesions. o Biphosphanates (eg, pamidronate [Aredia]), potent inhibitors of bone resorption, to treat hypercalcemia and alleviate bone pain. o Allopurinol (Zyloprim) and fluids to treat hyperuricemia. o Hemodialysis to manage renal failure. o Surgical stabilization and fixation of fractures. DRUG ALERT

Pamidronate and other biphosphanates may cause transient temperature elevations, hypophosphatemia, hypomagnesemia, hypocalcemia, and local reactions at the site of I.V. administration, such as thrombophlebitis, pain, and erythema. Biphosphanates are administered as I.V. infusions, generally during 4 or more hours; rapid I.V. administration may cause renal failure. Complications

Pathologic fractures, spinal cord compression. Recurrent infections, particularly bacterial. Electrolyte abnormalities (hypercalcemia, hypophosphatemia). Renal failure, pyelonephritis. Bleeding. Thromboembolic complications caused by hyperviscosity.
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Patients with multiple myeloma treated by chemotherapy have a median survival of 2 to 3 years; the impact of newer treatment options on survival is still unknown.

Nursing Assessment

Obtain health history, focusing on pain, fatigue. Evaluate for evidence of bone deformities and bone tenderness or pain. Assess patient's support system and personal coping skills.

Nursing Diagnoses

Acute and chronic pain (bone) related to destruction of bone and possible pathologic fractures Impaired Physical Mobility related to pain and possible fracture Fear related to poor prognosis Risk for Injury related to complications of disease process

Nursing Interventions Controlling Pain


Assess for presence, location, intensity, and characteristics of pain. Administer pharmacologic agents as ordered to control pain. Use adequate doses of regularly scheduled, around-the-clock analgesics. Teach the use of nonpharmacologic methods, such as the use of music, relaxation breathing, progressive muscle relaxation, distraction, and imagery, to help to manage pain. Assess effectiveness of analgesics and adjust dosage or drug used as necessary to control pain.

Promoting Mobility

Encourage patient to wear back brace for lumbar lesion. Recommend physical and occupational therapy consultation. Discourage bed rest to prevent hypercalcemia but ensure safety of environment to prevent fractures. Assist patient with measures to prevent injury and decrease risk of fractures. Advise avoidance of lifting and straining; use walker and other assistive devices as appropriate.

Relieving Fear
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Develop trusting, supportive relationship with patient and his significant others. Encourage patient to discuss medical condition and prognosis with health care provider when patient is ready. Assure patient that you are available for support, to provide comfort measures, and to answer questions. Encourage use of patient's own support network, religious and community services, and national agencies (see Box 26-1, page 952).

Monitoring for Complications


Report sudden, severe pain, especially of back, which could indicate pathologic fracture. Watch for nausea, drowsiness, confusion, polyuria, which could indicate hypercalcemia caused by bony destruction or immobilization. Monitor serum calcium levels. Check results of blood urea nitrogen (BUN) and creatinine and urine protein tests to detect renal insufficiency, caused by nephrotoxicity of abnormal proteins in multiple myeloma. Increase fluid intake, monitor intake and output, and weigh patient daily.

Community and Home Care Considerations


Make sure patient has appropriate housing and equipment to support decreased mobility and risk of pathologic fractures (eg, stair handrails, cane or walker, commode chair). Inspect home environment for throw rugs, cluttered furnishings, dark hallways, or difficult stairs that may cause a fall and possible fracture.

Patient Education and Health Maintenance


Teach patient about risk of infection caused by impaired antibody production and chemotherapy (see Patient Education Guidelines). Teach patient to take medications as prescribed and monitor for possible adverse effects; avoid aspirin and NSAIDs unless prescribed by health care provider because these drugs may interfere with platelet function. Teach patient to minimize risk of fractures. Use proper body mechanics and assistive devices as appropriate; avoid bed rest, remain ambulatory. Advise patient to report new onset of pain, new location, or sudden increase in pain intensity immediately. Report new onset or worsening of neurologic symptoms (eg, changes in sensation) immediately. Encourage the patient to maintain high fluid intake (2 to 3 L/day) to avoid dehydration and prevent renal insufficiency; also not to fast before diagnostic tests. Provide patient and family with information about resources in the community, such as the Leukemia and Lymphoma Society and the American Cancer Society (see Box 26-1).

Evaluation: Expected Outcomes


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States decreased pain Ambulates without injury Asks questions about disease; contacts support group No development of complications

BLEEDING DISORDERS Bleeding disorders may be congenital or acquired and may be caused by dysfunction in any phase of hemostasis (clot formation and dissolution). Bleeding disorders seen in adults include thrombocytopenia, idiopathic thrombocytopenic purpura (ITP), DIC, and von Willebrand's disease. THROMBOCYTOPENIA Thrombocytopenia is decreased platelet count (less than 100,000/mm3), the most common cause of bleeding disorders. Pathophysiology and Etiology Classification by Etiology

Decreased platelet production infiltrative diseases of bone marrow, leukemia, aplastic anemia, myelofibrosis, myelosuppressive therapy, radiation therapy; may include inherited disorders, such as Fanconi's anemia and Wiskott-Aldrich syndrome. Increased platelet destruction infection, drug-induced (eg, heparin), ITP, DIC. Abnormal distribution or sequestration in spleen. Dilutional thrombocytopenia after hemorrhage, RBC transfusions.

Clinical Manifestations

Usually asymptomatic. When platelet count drops below 20,000/mm3: o Petechiae occur spontaneously o Ecchymoses occur at sites of minor trauma (venipuncture, pressure) o Bleeding may occur from mucosal surfaces, nose, GI and GU tracts, respiratory system, and within CNS o Menorrhagia is common. Excessive bleeding may occur after procedures (dental extractions, minor surgery, biopsies). Thrombotic complications (arterial and venous) and areas of skin necrosis are associated with heparin-induced thrombocytopenia.

Diagnostic Evaluation

CBC with platelet count decreased hemoglobin, hematocrit, platelets. Bleeding time, prothrombin time (PT), partial thromboplastin time (PTT) prolonged.
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Platelet aggregation test for heparin-dependent platelet antibodies positive.

Management

Treat underlying cause. Platelet transfusions. Steroids or I.V. immunoglobulins may be helpful in selected patients. Heparin-induced thrombocytopenia: discontinue heparin, use alternate anticoagulant therapy due to high risk of thromboses (direct thrombin inhibitors, such as argatroban or hirudin), avoid platelet transfusions.

Complications Severe blood loss or bleeding into vital organs may be life-threatening. Nursing Assessment

Obtain health history, focusing on prior illnesses and episodes of bleeding, past surgical experiences, exposure to toxins or ionizing radiation, family history of bleeding. Obtain list of current and recent medications (including OTC preparations, herbal and dietary supplements). Perform complete physical examination for signs of bleeding.

Nursing Diagnosis

Risk for Injury related to bleeding due to thrombocytopenia

Nursing Interventions Minimizing Bleeding

Institute bleeding precautions. o Avoid use of plain razor, hard toothbrush or floss, I.M. injections, tourniquets, rectal procedures. suppositories. o Administer stool softeners as necessary to prevent constipation. 3 o Restrict activity and exercise when platelet count is less than 20,000/mm or when active bleeding occurs. Monitor pad count and amount of saturation during menses; administer or teach self-administration of hormones to suppress menstruation as prescribed. Administer blood products as ordered. Monitor for signs and symptoms of allergic reactions, anaphylaxis, and volume overload. Evaluate urine, stool, and emesis for gross and occult blood.
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Patient Education and Health Maintenance


Teach patient bleeding precautions (see Patient Education Guidelines). Demonstrate the use of direct, steady pressure at bleeding site if bleeding develops. Encourage routine follow-up for platelet counts.

Evaluation: Expected Outcomes

Episodes of bleeding rapidly controlled; platelet count maintained at goal (usually 20,000/mm3)

AUTOIMMUNE (IDIOPATHIC) THROMBOCYTOPENIC PURPURA Autoimmune thrombocytopenic purpura, or idiopathic immune thrombocytopenic purpura, is an acute or chronic bleeding disorder that results from immune destruction of platelets by antiplatelet antibodies. Pathophysiology and Etiology

Autoantibodies of both immunoglobulin (Ig) G and IgM subclasses, directed against a platelet-associated antigen, lead to destruction of platelets in spleen, liver. Acute disorder more common in childhood, typically following viral illness; has good prognosis with 80% to 90% recovering uneventfully. Typically lasts 1 to 2 months. Chronic disorder (more than 6-month course) most common between ages 20 and 40, three times more common in women, may last for years or even indefinitely. May be associated with pregnancy or with development of systemic lupus erythematosus or thyroid disease.

Clinical Manifestations Bruising, petechiae, bleeding from nares and gums, menorrhagia. Diagnostic Evaluation

CBC demonstrates platelet count less than 20,000/mm3 (acute ITP); 30,000 to 70,000/mm3 (chronic ITP); may also be lymphocytosis and eosinophilia. Bone marrow aspirate shows increased numbers of young megakaryocytes, sometimes increased numbers of eosinophils. Assay for platelet autoantibodies sometimes helpful.

Management

Supportive care: judicious use of platelet transfusions, control of bleeding.


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High-dose corticosteroids, I.V. immunoglobulins, parenteral anti-D (for Rhesus positive patients with spleens), azathioprine (Imuran), cyclophosphamide (Cytoxan), vincristine (Oncovin). Splenectomy removes potential site for sequestration and destruction of platelets.

Complications Severe blood loss or bleeding into vital organs may be life-threatening. Nursing Assessment

Obtain history of bleeding episodes, including bruising and petechiae, bleeding of gums, and heavy menses. Perform physical examination for signs of bleeding.

Nursing Diagnosis

Risk for Injury related to bleeding due to thrombocytopenia

Nursing Interventions Minimizing Bleeding


Institute bleeding precautions. Monitor pad count and amount of saturation during menses; administer or teach self-administration of hormones to suppress menstruation as prescribed. Administer blood products as ordered. Monitor for signs and symptoms of allergic reactions, anaphylaxis, and volume overload. Evaluate all urine and stools for gross and occult blood.

Patient Education and Health Maintenance


Teach patient bleeding precautions (see Patient Education Guidelines). Demonstrate the use of direct, steady pressure at bleeding site if bleeding does develop. Encourage routine follow-up for platelet counts.

Evaluation: Expected Outcomes

Episodes of bleeding rapidly controlled


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DISSEMINATED INTRAVASCULAR COAGULATION DIC is an acquired thrombotic and hemorrhagic syndrome characterized by abnormal activation of the clotting cascade and accelerated fibrinolysis. This results in widespread clotting in small vessels with consumption of clotting factors and platelets, so that bleeding and thrombosis occur simultaneously. Pathophysiology and Etiology

A syndrome arising with an underlying disorder or event: o Overwhelming infections, particularly bacterial sepsis. o Obstetric complications: abruptio placentae, eclampsia, amniotic fluid embolism, retention of dead fetus. o Massive tissue injury: burns, trauma, fractures, major surgery, fat embolism, organ destruction (eg, severe pancreatitis, hepatic failure). o Vascular and circulatory collapse, shock. Hemolytic transfusion reaction. Snake bites. Recreational drugs. Malignancy: particularly lung, colon, stomach, pancreas.

Clinical Manifestations

Signs of abnormal clotting: o Coolness and mottling of extremities. o Acrocyanosis (cold, mottled extremities with clear demarcation from normal tissue). o Dyspnea, adventitious breath sounds. o Altered mental status. o Acute renal failure. o Pain (eg, related to bowel infarction). Signs of abnormal bleeding: o Oozing, bleeding from sites of procedures, I.V. catheter insertion sites, suture lines, mucous membranes, orifices. o Internal bleeding leading to changes in vital organ function, altered vital signs.

Diagnostic Evaluation

Platelet count diminished. PT, PTT, and thrombin time prolonged. Fibrinogen decreased level.
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Fibrin split (degradation) products increased level. D-dimer fibrin degradation product increased level. Antithrombin III decreased level. Protein C decreased level.

Management

Treat underlying disorder. Replacement therapy for serious hemorrhagic manifestations: o Fresh-frozen plasma replaces clotting factors. o Platelet transfusions. o Cryoprecipitate replaces clotting factors and fibrinogen. Supportive measures including fluid replacement, oxygenation, maintenance of BP and renal perfusion. Heparin therapy (controversial) inhibits clotting component of DIC.

Complications

Thromboembolic: pulmonary embolism; cerebral, myocardial, splenic, or bowel infarction; acute renal failure; tissue necrosis or gangrene. Hemorrhagic: cerebral hemorrhage is most common cause of death in DIC.

Nursing Assessment

Be aware that seriously ill patients are at risk; monitor condition closely. Assess for signs of bleeding and thrombosis, including chest pain, shortness of breath, hematuria, abdominal pain, headache, numbness and coolness of an extremity.

Nursing Diagnoses

Risk for Injury related to bleeding due to thrombocytopenia Ineffective Tissue Perfusion (all tissues) related to ischemia due to microthrombi formation

Nursing Interventions Minimizing Bleeding

Institute bleeding precautions.


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Monitor pad count and amount of saturation during menses; administer or teach self-administration of hormones to suppress menstruation as prescribed. Administer blood products as ordered. Monitor for signs and symptoms of allergic reactions, anaphylaxis, and volume overload. Avoid dislodging clots. Apply pressure to sites of bleeding for at least 20 minutes, use topical hemostatic agents. Use tape cautiously. Maintain bed rest during bleeding episode. If internal bleeding is suspected, assess bowel sounds and abdominal girth. Evaluate fluid status and bleeding by frequent measurement of vital signs, central venous pressure, intake and output.

Promoting Tissue Perfusion


Keep patient warm. Avoid vasoconstrictive agents (systemic or topical). Change patient's position frequently and perform range of motion exercises. Monitor electrocardiogram and laboratory tests for dysfunction of vital organs caused by ischemiaarrhythmias, abnormal arterial blood gas levels, increased BUN and creatinine. Monitor for signs of vascular occlusion and report immediately. o Brain decreased LOC, sensory and motor deficits, seizures, coma. o Eyes visual deficits. o Bone bone pain. o Pulmonary vasculature chest pain, shortness of breath, tachycardia. o Extremities cold, mottling, numbness. o Coronary arteries chest pain, arrhythmias. o Bowelpain, tenderness, decreased bowel sounds.

Patient Education and Health Maintenance Explain the syndrome and its management to patient and family members as part of reassurance and support during this critical illness. Evaluation: Expected Outcomes

Episodes of bleeding rapidly controlled Alert, vital signs stable, urine output adequate, no complaints of chest pain or shortness of breath

VON WILLEBRAND'S DISEASE Inherited (autosomal dominant) or acquired bleeding disorder characterized by decreased level of von Willebrand factor and prolonged bleeding time. Pathophysiology and Etiology
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von Willebrand factor synthesized in vascular endothelium, megakaryocytes and platelets; enhances platelet adhesion as first step in clot formation, also acts as carrier of factor VIII in blood. von Willebrand's is most common inherited bleeding disorder; includes multiple subtypes with varying severity. Acquired form is rare, generally appears late in life, typically in association with lymphoma, leukemia, multiple myeloma, or autoimmune disorder.

Clinical Manifestations

Mucosal and cutaneous bleeding (eg, bruising, gingival bleeding, epistaxis, menorrhagia). Prolonged bleeding from cuts or after dental and surgical procedures.

Diagnostic Evaluation

Bleeding time prolonged. Ristocetin cofactor abnormal. von Willebrand's factor decreased. von Willebrand's factor multimers demonstrate defective von Willebrand's factor in some types. Factor VIII generally decreased.

Management

Replacement of von Willebrand's factor and factor VIII using clotting factor concentrates (Alphanate, Humate-P). Antifibrinolytic medications (Amicar, tranexamic acid) to stabilize clot formation before dental procedures and before minor surgery. Desmopressin acetate (DDAVP), a synthetic analogue of vasopressin, may be used to manage mild to moderate bleeding. Estrogen and progesterone stimulate production of von Willebrand's factor and Factor VIII and may be particularly helpful in control of menorrhagia.

Complications Severe blood loss or bleeding into vital organs may be life-threatening. Nursing Assessment

Obtain history of bleeding episodes such as menstrual flow. Ask quantitative questions (eg, how many nosebleeds do you have each year?) as patient may not realize his experience is abnormal. Perform physical examination for signs of bleeding.
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Nursing Diagnosis

Risk for Injury related to bleeding due to decreased level of von Willebrand's factor and factor VIII

Nursing Interventions Minimizing Bleeding

Institute bleeding precautions: o Avoid use of plain razor, hard toothbrush or floss. o Avoid intramuscular injections, tourniquets, rectal procedures or suppositories. o Administer stool softeners as necessary to prevent constipation. 3 o Restrict activity and exercise when platelet count less than 20,000/mm or when active bleeding occurs. Monitor pad count and amount of saturation during menses; administer or teach self-administration of hormones to suppress menstruation as prescribed. Administer blood products as ordered. Monitor for signs and symptoms of allergic reactions, anaphylaxis, and volume overload. Use topical hemostatic agents, such as absorbable gelatin (Gelfoam), oxidized cellulose (Surgicel), topical adrenaline, or phenylephrine, if pressure and use of ice does not stop bleeding.

Patient Education and Health Maintenance


Teach patient bleeding precautions (see Patient Education Guidelines,). Demonstrate the use of direct, steady pressure at bleeding site if bleeding develops.

Evaluation: Expected Outcomes

Episodes of bleeding rapidly controlled

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