Assessment of Hematologic Disorders
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Questions and Answers

Which condition is characterized by a reduction in platelet count and presents with manifestations such as bruising and bleeding?

  • Hemophilia
  • Leukemia
  • Immune Thrombocytopenic Purpura (ITP) (correct)
  • Von Willebrand Disease
  • What is a common medical management approach for patients with Hemophilia?

  • Corticosteroids
  • Antibiotic therapy
  • Platelet transfusions
  • Factor replacement therapy (correct)
  • Which clinical manifestation might most commonly be observed in a patient with Leukemia?

  • Increased infection rates (correct)
  • Fluid retention
  • Excessive bruising
  • Joint swelling
  • In Immune Thrombocytopenic Purpura, which of the following is a potential cause of low platelet counts?

    <p>Increased platelet destruction</p> Signup and view all the answers

    Which of the following disorders primarily affects the ability of blood to clot normally?

    <p>Hemophilia</p> Signup and view all the answers

    What is a characteristic clinical feature of Immune Thrombocytopenic Purpura (ITP)?

    <p>Spontaneous mucosal bleeding</p> Signup and view all the answers

    Which laboratory test is most indicative of Hemophilia?

    <p>Prolonged activated partial thromboplastin time (aPTT)</p> Signup and view all the answers

    Which of the following is a common complication associated with Leukemia?

    <p>Neutropenic fever</p> Signup and view all the answers

    What is the primary target of treatment in Immune Thrombocytopenic Purpura (ITP)?

    <p>Reduction of platelet destruction</p> Signup and view all the answers

    What is a potential ethical consideration in the management of patients with Hemophilia?

    <p>Avoiding prophylactic treatment when warranted</p> Signup and view all the answers

    What is the primary demographic trend associated with Immune Thrombocytopenic Purpura (ITP)?

    <p>It is more frequent in children and young women.</p> Signup and view all the answers

    How does Immune Thrombocytopenic Purpura (ITP) generally present across different age groups?

    <p>It can affect individuals of all ages, with varied severity.</p> Signup and view all the answers

    Which of the following statements about the occurrence of Immune Thrombocytopenic Purpura (ITP) is inaccurate?

    <p>Only women in their 20s experience ITP frequently.</p> Signup and view all the answers

    What underlying trend characterizes the incidence of Immune Thrombocytopenic Purpura (ITP) in young populations?

    <p>It increases significantly after puberty.</p> Signup and view all the answers

    What age group experiences the highest incidence of Immune Thrombocytopenic Purpura (ITP)?

    <p>Children and teenagers.</p> Signup and view all the answers

    Signup and view all the answers

    Study Notes

    Assessment and Management of Patients With Hematologic Disorders

    • This topic covers assessment and management of patients with immune thrombocytopenic purpura (ITP), hemophilia, and leukemia.

    Learning Objectives

    • Identify clinical manifestations and medical management for ITP, hemophilia, and leukemia.
    • Apply the nursing process as a framework for patient care with these conditions.

    Thrombocytopenia

    • Low platelet level caused by several factors.
    • Decreased platelet production in the bone marrow (e.g., aplastic anemia, hematological malignancy, chemotherapy, radiation).
    • Increased platelet destruction (e.g., ITP, Lupus erythematosus, sequestration in enlarged spleen).
    • Increased platelet consumption (e.g., clot formation in major bleeding).

    Immune Thrombocytopenic Purpura (ITP)

    • A disease affecting people of all ages, more common in children and young women.
    • Also known as idiopathic thrombocytopenic purpura or immune thrombocytopenia.
    • Primary ITP has no identifiable underlying cause.
    • Secondary ITP often results from autoimmune diseases (e.g., antiphospholipid antibody syndrome), viral infections (e.g., hepatitis C, HIV), and various drugs (e.g., sulfa drugs).

    Clinical Manifestations of ITP

    • Many patients have no symptoms.
    • Easy bruising, heavy menses, and petechiae (small red spots) on extremities or trunk.
    • "Dry purpura": simple bruising or petechiae.
    • "Wet purpura": bleeding from mucosal surfaces like the GI tract (mouth, hemoptysis) or pulmonary system indicating a higher risk of life-threatening bleeding.
    • Severe thrombocytopenia (platelet count < 20,000/mm³).

    Assessment and Diagnostic Findings for ITP

    • Obtain a careful history and physical assessment.
    • Test for hepatitis C and HIV.
    • Perform a bone marrow aspirate.
    • A platelet count < 20,000/mm³ and prolonged bleeding time suggest ITP.

    Medical Management of ITP

    • Immunosuppressive agents (e.g., corticosteroids) are the mainstay of short-term therapy. Typical dose is 1 mg/kg body weight for 21 days, then tapered.
    • Intravenous immunoglobulin (IVIG) is commonly used.
    • Splenectomy (spleen removal) may be considered if other treatments are ineffective.

    Nursing Management of ITP

    • Assess patient lifestyle to determine bleeding risk.
    • Obtain a thorough medication history (including OTC, herbs, and supplements).
    • Monitor for sulfa-containing medications and other drugs that impair platelet function (e.g., aspirin, NSAIDs).
    • Avoid injections and rectal medications.
    • Avoid rectal temperature measurements.
    • Educate patients about signs of exacerbation (e.g., petechiae, ecchymoses).
    • Educate on current treatment, side effects, tapering schedules, and platelet count monitoring frequency.
    • Advise patients to avoid constipation and the Valsalva maneuver, and vigorous toothbrushing.
    • Recommend electric razors and soft-bristled toothbrushes.
    • Monitor bone mineral density and consider calcium and vitamin D supplementation if needed (long-term steroid use).

    Hemophilia

    • Two inherited bleeding disorders (A and B) that are clinically indistinguishable but distinguished by laboratory tests.
    • Hemophilia A (Classical hemophilia) is due to a genetic defect causing factor VIII deficiency or malfunction.
    • Hemophilia B (Christmas disease) is due to a genetic defect causing factor IX deficiency or malfunction.
    • Hemophilia A is about five times more common than hemophilia B.

    Clinical Manifestations of Hemophilia

    • Bleeding into various parts of the body, potentially severe even with minimal trauma.
    • Frequency and severity depend on the degree of factor deficiency and the intensity of precipitating trauma.
    • Spontaneous hemorrhages, particularly hemarthroses (bleeding in joints), hematomas, hematuria (blood in urine), and GI bleeding may occur.
    • Recurrent hemarthroses lead to chronic pain, ankylosis(joint immobility), or arthropathy (joint damage).

    Diagnostic Tests for Hemophilia

    • Prolonged partial thromboplastin time (PTT) is a sign.
    • Determine the levels of clotting factors to identify which factor is deficient.

    Medical Management of Hemophilia

    • Hemophilia is not curable.
    • Treatment focuses on stopping bleeding episodes by increasing the blood levels of the missing clotting factor (replacement therapy).
    • Severe hemophilia A is treated with factor VIII; hemophilia B is treated with factor IX.

    Nursing Management of Hemophilia

    • During bleeding episodes, administer clotting factor or plasma as ordered.
    • Repeat infusions until bleeding stops.
    • Apply cold compresses and elevate affected areas.
    • Restrict patient activity for 48 hours after bleeding stops.
    • Elevate affected joints if bleeding occurs within the joint.
    • Control pain with analgesics as prescribed.
    • Avoid aspirin and aspirin-containing medications due to their impact on platelet function.

    Leukemia

    • A neoplastic proliferation of a specific blood cell type (granulocytes, lymphocytes, or erythrocytes).

    Classifications

    • Leukemia is categorized by the involved stem cell line (lymphoid and myeloid).
    • Leukemia is also categorized as acute or chronic based on how quickly the disease develops.

    Pathophysiology of Leukemia

    • Immature blood cells proliferate in the bone marrow, hindering the development of normal blood cells.
    • Accumulation of abnormal cells in the peripheral circulation and body tissues/organs.
    • Normal hematopoiesis is inhibited, reducing mature blood cell counts (granulocytes, erythrocytes, and platelets).

    Acute Lymphocytic Leukemia (ALL)

    • A common childhood cancer characterized by abnormal growth of lymphocyte precursors.

    • Common in children, with a sudden onset of high fever, abnormal bleeding, petechiae, ecchymoses, and easy bruising.

    Chronic Lymphocytic Leukemia (CLL)

    • Primarily affects B and T lymphocytes in adults.
    • Develops in a three-phase process; the first (insidious) phase is characterized by anemia and mild bleeding abnormalities.

    Risk Factors of Leukemia

    • Certain viruses.
    • Genetic and immunological factors.
    • Exposure to radiation, chemicals, and drugs.
    • Environmental factors.

    Clinical Manifestations of Leukemia

    • Low-grade fever (infection).
    • Pallor, weakness, lethargy, shortness of breath, and malaise from anemia.
    • Fatigue, tachycardia, palpitations, and abdominal pain.
    • Sternal pain and rib tenderness from bone marrow crowding.
    • Confusion, headaches, personality changes with central nervous system involvement.
    • Ecchymosis or petechiae (low platelets).

    Diagnostic Tests for Leukemia

    • Bone marrow aspiration to evaluate the degree of malignant white blood cell proliferation.
    • Lumbar puncture to rule out involvement of the central nervous system.
    • Genetic analysis of peripheral blood and bone marrow components.
    • Complete blood count (CBC) to assess platelets, red blood cells, and mature white blood cells.

    Therapeutic Measures for Leukemia

    • Systemic chemotherapy to eradicate leukemic cells and induce remission.
    • Radiation therapy.
    • Bone marrow transplant (BMT).
    • Peripheral blood stem cell transplantation.
    • Biological therapies.

    Nursing Care Plan for Leukemia

    • Risk of injury (infection, bleeding) related to pancytopenia.
    • Impaired oral mucosa from chemotherapy or antimicrobials.
    • Pain and discomfort related to mucositis, fever, and infection.
    • Imbalanced nutrition (less than body requirements).
    • Fatigue and activity intolerance.
    • Self-care deficits (bathing, dressing, toileting).
    • Anxiety and grief.
    • Deficient knowledge about the disease process, treatment, complications, and self-care.

    Prevention of Infection in Leukemia

    • Avoid crowds and people who are ill.
    • Do not share personal items.
    • Wash hands frequently and thoroughly with antimicrobial soap.
    • Monitor and record temperature.
    • Report any signs of infection (fever >100˚F, cough, purulent drainage from open sores, abscesses) to the doctor immediately.

    Maintaining Intact Oral Mucosa

    • Encourage and assist with mouth care.
    • Avoid oral irritants (commercial mouthwashes, alcohol, tobacco).
    • Brush teeth using a soft-bristled toothbrush and non-abrasive toothpaste.
    • Use oral ice chips and maintain adequate hydration.
    • Use normal saline mouth rinses every 1-4 hours.
    • Avoid spicy or hard-to-chew foods with extreme temperatures.
    • Apply water-soluble lip balm.

    Improving Nutritional Intake

    • Encourage and provide mouth care before and after meals.
    • Administer analgesics before eating to improve appetite.
    • Offer small, frequent meals with soft textures and moderate temperatures.
    • Provide nutritional supplements as needed.
    • Monitor daily body weight.
    • Parenteral nutrition may be required.

    Easing Pain and Discomfort

    • Treat recurrent fevers and myalgias/arthralgias with appropriate analgesics.
    • Use cool water compresses (avoid ice).
    • Gentle back and shoulder massage.
    • Patient-controlled analgesia may be helpful.
    • Active listening and referral for professional counseling when appropriate.

    Maintaining Fluid and Electrolyte Balance

    • Monitor intake and output.
    • Monitor daily weight.
    • Evaluate for signs of dehydration or fluid overload.
    • Monitor electrolytes including potassium and magnesium.
    • Monitor laboratory tests (including blood urea nitrogen, creatinine, and hematocrit).

    Managing Anxiety and Grief

    • Provide emotional support and discuss the uncertain future.
    • Assess how much information the patient wants to receive.
    • Recognize and address depressive symptoms and grief related to losses (family, professional, social, and physical role functions).
    • Support role restructuring as needed.
    • Encourage the patient to prioritize their decisions and take time where needed.

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    Description

    This quiz focuses on the assessment and management of patients with hematologic disorders, including immune thrombocytopenic purpura (ITP), hemophilia, and leukemia. You will learn to identify clinical manifestations and apply the nursing process for effective patient care.

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