Notes on Hematologic Disorders and Patient Care

Chapter 16: Care for Patients With Hematologic Disorders

Lesson Objectives

• Theory Objectives

  • Examine the causes of various types of anemias.

  • Develop a plan of care for patients with anemia.

  • Explain the pathophysiology and care of sickle cell disease.

  • Compare cell abnormalities of polycythemia vera with those of leukemia.

  • Formulate a teaching plan for the patient with leukemia.

  • Interpret lab values for a patient with leukemia.

  • Summarize the problems and treatments faced by patients with hemophilia.

    • Clinical Practice Objectives

  • Write expected outcomes for nursing diagnoses for patients with blood disorders, considering goals of care.

  • Prepare for pre-procedure and post-procedure care for patients undergoing bone marrow aspiration.

  • Perform assessments for patients with suspected hematologic disorders.

  • Assist in developing a care plan for adults with leukemia.

  • Assess for signs and symptoms of disseminated intravascular coagulation (DIC).

Anemia

Etiology
  • Anemia can be attributed to:
    • Blood loss
    • Failure in blood cell production
    • Excessive destruction of red blood cells
Pathophysiology
  • Anemia occurs when total body iron is insufficient and erythropoiesis (the production of red blood cells) is diminished.
  • A deficiency of iron inhibits the formation of hemoglobin (Hb), which is crucial for transporting oxygen.
Specific Mechanism of Iron Deficiency Anemia
  • Increased blood loss or RBC destruction, along with decreased RBC production, leads to:
    • A reduced oxygen-carrying ability of blood.
    • Tissue hypoxia resulting in symptoms.
Signs and Symptoms

  • Mild Anemia (Hb range: 11 to 13 g/dL)

    • Headaches
    • Palpitations
    • Dyspnea on exertion

  • Moderate Anemia (Hb range: 8 to 10 g/dL)

    • Brittle nails
    • Sore tongue
    • Pallor
    • Chronic fatigue
    • Headaches
    • Dizziness or faintness

  • Severe Anemia (Hb < 8 g/dL)

    • General signs and symptoms affecting Eyes, Skin, Cardiovascular, Respiratory, Gastrointestinal, Musculoskeletal, and Neurological systems.
Treatment Options
  • Iron supplementation
  • Vitamin supplementation
  • Dietary adjustments
  • Blood transfusion, particularly Vitamin B12 if deficient

Aplastic Anemia

  • Etiology

    • May develop after viral infections, drug reactions, or inherited tendencies.

  • Pathophysiology

    • Characterized by bone marrow depression, often considered an immune-mediated disease, leading to decreased levels of red cells, white cells, and platelets.

  • Treatment involves eliminating any identifiable underlying causes and may require administration of packed red cells and platelets, as well as antibiotics for infections.

Sickle Cell Disease

Etiology
  • A genetic disorder leading to the production of hemoglobin S.
Pathophysiology
  • Hypoxia causes hemoglobin S to assume a sickle shape, leading to blocked vessels and thrombus formation.
Signs and Symptoms
  • Pallor, lethargy, and pain; painful swelling of hands and feet due to bone infarction.
Treatment
  • Prevention of sickle cell crisis and management of symptoms during crises.
  • Encourage regular intake of folic acid and a protein-rich diet, prevent infections, ensure adequate fluid intake, and avoid alcohol and recreational drugs.
  • Hydroxyurea (Hydrea) can decrease the frequency of sickling episodes.
Nursing Management
  • Provide patient education on:
    • Avoiding high altitudes, vigorous exercise, and iced liquids.
    • Maintaining adequate fluid intake and refraining from smoking.
    • Prompt treatment of infections and adequate rest.
    • Pain relief and monitoring intake/output.
    • Oxygen therapy as required.

Polycythemia Vera

  • A neoplastic disorder characterized by overproduction of red blood cells, resulting in thickened blood that leads to sluggish blood flow and distended blood vessels.
Treatment Options
  • Phlebotomy, antineoplastic agents, and radiation therapy.
  • Increased fluid intake and use of aspirin to manage symptoms.

Leukemia

Etiology
  • Though classified as a cancer, the exact cause of leukemia is not known.
Pathophysiology
  • Immature cells in the blood interfere with the production of healthy cells.
Signs and Symptoms
  • Fever, headaches, bone pain, pallor, weakness, fatigue, malaise, recurrent infections, swollen lymph nodes, enlarged spleen, unexpected weight loss, and easy bleeding or thrombosis.
Treatment
  • Aim to slow down the growth of malignant blood cells, maintain normal levels of red cells, hemoglobin, and platelets, symptom management, and exploring potential curative therapies such as hematopoietic cell transplantation (HCT).
Nursing Management
  • Address potential infections, abnormal bleeding, anemia, nutritional alterations due to loss of appetite, elevated uric acid levels from chemotherapy, and psychosocial issues related to disease impact and treatment.

Thrombocytopenia

Causes
  • Bone marrow depression from chemotherapy/radiation, autoimmune diseases, infections (bacterial/viral), DIC, or splenic over-function.
Nursing Care
  • Focus on preventing bleeding and close observation for spontaneous bleeding and prompt intervention.
  • Invasive procedures should only be performed when necessary, and patients must avoid activities that could induce bleeding.
Safety Alert: Prevent Bleeding
  • When performing venipuncture, injections, or discontinuing catheters/needles in patients with a low platelet count, maintain pressure over the site for at least 10 minutes to prevent ongoing oozing.

Multiple Myeloma

Etiology and Pathophysiology
  • Characterized by uncontrolled multiplication of abnormal plasma cells in the bone marrow leading to excessive production of abnormal immune globulin and cytokines.
Signs and Symptoms
  • Symptoms appear gradually and typically when the skeletal system is significantly involved; these may include backaches, bone pain, pathologic fractures, and severe pain.
Treatment
  • Involves chemotherapy, radiation, hematopoietic cell transplantation (HCT), pain management, and strategies to prevent pathologic fractures.
Nursing Management
  • Provide supportive care for complications from the disease and treatments.
  • Encourage hydration (3 to 5 L/day) to address hypercalcemia and manage pain using acetaminophen, NSAIDs, or narcotics.
  • Exercise caution when moving patients due to fracture risk.

Hemophilia

Etiology
  • An inherited X-linked disorder characterized by a deficiency of specific clotting factors.
Pathophysiology
  • There is a decrease in the activity of one of the 11 clotting factors necessary for proper clot formation.
Signs and Symptoms
  • Bleeding tendencies and hemarthrosis (bleeding into joints).
Diagnosis
  • Involves history, physical exam, complete blood count (CBC), coagulation profiles, and tests for clotting factors.
Treatment
  • Includes recombinant forms of factor VIII and IX, desmopressin acetate (DDAVP), tranexamic acid (Cyklokapron), aminocaproic acid (Amicar), along with analgesics and corticosteroids.
Nursing Management
  • Administer necessary clotting factors, elevate injured body parts, apply cold packs, control pain, observe for additional bleeding, provide psychological support, and encourage genetic counseling for the family if not previously offered.

Disseminated Intravascular Coagulation (DIC)

  • A complex disorder typically associated with tissue destruction, where tissue thromboplastin is released, causing excessive clotting throughout the microcirculation.
  • Treatment focuses on correcting underlying issues (e.g., trauma, infection).
Nursing Management
  • Monitor for signs of DIC in at-risk patients, detect external bleeding early, and monitor sensorium and vital signs for indications of internal bleeding.

Blood Transfusions

  • involve administering a component of blood, for which consent is required. Blood products require verification by two nurses before administration.

Signs and Symptoms of a Transfusion Reaction

  • Symptoms may include chills, fever, shortness of breath, itching or rash, sense of impending doom, headache, pain in the low back or chest, tachycardia, tachypnea, hypotension, hemoglobinuria, and shock.
Types of Acute Transfusion Reactions
  • Acute hemolytic reaction
  • Febrile, non-hemolytic reaction (most common)
  • Mild allergic reaction
  • Anaphylactic and severe allergic reaction
  • Circulatory overload
  • Sepsis

Management of Hematologic Disorders

  • Include oxygen therapy, iron therapy, vitamin B12 therapy, lumbar puncture in emergencies for trauma to the spleen, and splenomegaly from blood disorders like leukemia.