Assessment and Management of Patients With Hematologic Disorders

1. Assessment and Management of Patients With Hematologic Disorders

2. Hematologic System • The blood and the blood forming sites, including the bone marrow and the reticuloendothelial system • Blood • Plasma (fluid portion of blood -55%) • Blood cells • Hematopoiesis • The body replenishes its supply of blood cells • Primary site is bone marrow

3. Blood Cells • Erythrocyte: RBC • Leukocyte: WBC • Neutrophil • Monocyte • Eosinophil • Basophil • Lymphocyte: T lymphocyte and B lymphocyte • Thrombocyte: platelet

4. Hematologic Studies • CBC • Peripheral smear • Bone marrow aspiration and biopsy

5. Blood Smear

6. Hemostasis

7. Polycythemia • Increased volume of erythrocytes (HCT >55%M/>50%F) • Primary • Proliferative disorder - bone marrow is hypercellular • Patients at increased risk for thromboses and bleeding (due to platelet dysfunction) • Managed usually via phlebotomy • Secondary • Caused by excessive prodcution of erythropoietin • May be d/t hypoxic stimulus, neoplasms (epo produced by malignancy)

8. Polycythemia • Manifestations • Hypervolemia and hyperviscosity • Ruddy complexion • Hypertension • Headache • Dizziness, tinnitus • Signs of embolization • Management • Phlebotomy - reduce HCT to 45% or less • Hydration • Avoid iron supplementation

9. Neutropenia • Absolute neutrophil count • WBC x %neutrophils • <1500/ ul (severe if <500/ul) • Predisposed to infection • Neutropenia may mask clasic signs of infection - e.g., even low grade fever may be of great significance • May progress rapidly to sepsis • Nursing and collaborative care • Determine cause • Identify offending organism • Institute prophylactic or therapeutic antibiotic • Administer hematopoetic growth factors • Institute protective environmental measures • See table 31-22 and 31-23

10. Leukemia • Hematopoietic malignancy with unregulated proliferation of leukocytes • Affect blood and blood-forming tissues of bone marrow, lymph system, and spleen • Types (see table 31-24) • Acute myeloid leukemia • Chronic myeloid leukemia • Acute lymphocytic leukemia • Chronic lymphocytic leukemia

11. Acute Myeloid Leukemia (AML) • Defect in the stem cells that differentiate into all myeloid cells: monocytes, granulocytes, erythrocytes, and platelets • Most common leukemia in adults • Affects all ages with peak incidence at age 60 • Abrupt onset • Manifestations: fever and infection, weakness and fatigue, bleeding tendencies, pain from enlarged liver or spleen, hyperplasia of gums, and bone pain • Treatment is aggressive chemotherapy: induction therapy, BMT, and PBSCT

12. Chronic Myeloid Leukemia (CML) • Mutation in myeloid stem cell with uncontrolled proliferation of cells: excessive neoplastic granulocyts • Stages: chronic phase, blast crisis • Uncommon in people under 20; incidence increases with age; mean age is 55 to 60 years • Life expectancy is 3 to 5 years • Manifestations (initially may be asymptomatic): malaise; anorexia; weight loss; sternal pain; enlarged, tender spleen; enlarged liver • Treatment: chemotherapy, BMT, and PBSCT, radiation

13. Acute Lymphocytic Leukemia • Uncontrolled proliferation of immature cells from lymphoid stem cell • Most common in young children • Prognosis is good for children; 80% event-free after 5 years, but survival drops with increased age • Manifestations: fever, bleeding, anemia, mouth sores, symptoms of meningeal involvement and liver, spleen, and bone marrow pain • Treatment: chemotherapy, BMT or PBSCT

14. Chronic Lymphocytic Leukemia • Malignant B lymphocytes, most of which are mature, may escape apoptosis, resulting in excessive accumulation of cells • Most common form of chronic leukemia in adults • More common in older adults and affects men more often • Survival varies from 2 to 14 years depending upon stage

15. Chronic Lymphocytic Leukemia (cont.) • Manifestations: lymphadenopathy, fatigue, anorexia, hepatomegaly, splenomegaly; in later stages, anemias and thrombocytopenia;; “B symptoms” include fever, night sweats, and weight loss • Treatment: early stage may require no treatment, chemotherapy,radiation

16. Chemotherapy • Induction therapy • Nursing implications • Intensification therapy • Consolidation therapy • Maintenance therapy

17. Nursing Process—Assessment of the Patient With Leukemia • Health history • Assess for symptoms of leukemia and complications of anemia, infection, and bleeding • Weakness and fatigue • See table 31-27 • Laboratory tests • Leukocyte count, ANC, hematocrit, platelets, electrolytes, and cultures reports

18. Nursing Process—Diagnosis of the Patient With Leukemia • Risk for bleeding • Risk for impaired skin integrity • Impaired gas exchange • Impaired mucous membrane • Imbalanced nutrition • Acute pain • Fatigue and activity intolerance • Risk for infection

19. Nursing Process—Diagnosis of the Patient With Leukemia (cont.) • Risk for excess fluid volume • Diarrhea • Risk for deficient fluid volume • Self-care deficit • Anxiety • Disturbed body image • Potential for spiritual distress • Grieving diagnoses • Deficient knowledge

20. Collaborative Problems/Potential Complications • Infection • Bleeding • Renal dysfunction • Tumor lysis syndrome • Nutritional depletion • Mucositis • Depression

21. Nursing Process—Planning the Care of the Patient With Leukemia • Major goals include absence of complications, attainment and maintenance of adequate nutrition, activity tolerance, ability for self-care and to cope with the diagnosis and prognosis, positive body image, and an understanding of the disease process and its treatment

22. Interventions • Reduce risk of infection • Reduce risk of bleeding • Mucositis • Frequent, gentle oral hygiene • Soft toothbrush, or if counts are low, sponge-tipped applicators • Rinse only with NS, NS and baking soda, or prescribed solutions • Perineal and rectal care

23. Improving Nutrition • Provide oral care before and after meals • Administer analgesics before meals • Provide appropriate treatment of nausea • Provide small, frequent feedings with soft foods that are moderate in temperature • Provide a low-microbial diet • Provide nutritional supplements

24. Lymphoma • Neoplasm originating in the bone marrow and lymphatic structures resulting in the proliferation of lymphocytes • Hodgkin’s lymphoma • Non-Hodgkin’s lymphoma

25. Hodgkin’s Disease • Unicentric origin • Reed–Sternberg cell • Suspected viral etiology (EBV); familial pattern; incidence occurs in early 20s and again after age 50 • Excellent cure rate with treatment • Manifestations: painless lymph node enlargement; pruritus; B symptoms : fever, night sweats, and weight loss • Treatment is determined by stage of the disease and may include chemotherapy and/or radiation therapy

26. Non-Hodgkin's Lymphoma (NHL) • Lymphoid tissues become infiltrated with malignant cells that spread unpredictably; localized disease is rare • Incidence increases with age; the average age of onset is 50 to 60 • Prognosis varies with the type of NHL • Diagnosed via lymph node biopsy and imaging • Treatment is determined by type and stage of disease and may include interferon, chemotherapy, and/or radiation therapy

27. Multiple Myeloma • Malignant disease of plasma cells in the bone marrow with destruction of bone • M protein and Bence-Jones protein • Median survival is 3 to 5 years; there is no cure • Manifestations: insidious onset; bone pain, osteoporosis, fractures, elevated serum protein hypercalcemia, renal failure, symptoms of anemia, fatigue, weakness, increased serum viscosity, and increased risk for bleeding and infection • Treatment may include chemotherapy, corticosteroids, stem cell transplant, and biphosphonates

28. Multiple Myeloma • Nursing care is relates to bone involvement and sequelae from bone breakdown • Hypercalcemia • Fractures • Pain • Infection

29. Bleeding Disorders • Thrombocytopenia • Idiopathic thrombocytopenia purpura (ITP) • Hemophilia • Acquired coagulation disorders: liver disease, anticoagulants, and vitamin K deficiency • Disseminated intravascular coagulation (DIC) • Bleeding precautions • See Nursing care plan 31-2, table 31-15

30. Hemophilia • Inherited bleeding disorder, results in deficiency of factor VIII or IX; varying degrees of disease • Manifested by hemorrhage into various body parts • Medical management includes FFP transfusion, recombinant clotting factors, desmopressin (DDAVP) • Nursing management: control bleeding, patient education aimed at prevention of bleeding

31. Disseminated Intravascular Coagulation • Not a disease but a sign of an underlying disorder; bleeding/thrombotic disorder • Severity is variable; may be life-threatening • Triggers may include sepsis, trauma, shock, cancer, abruptio placentae, toxins, and allergic reactions, malignancies, liver disease • Altered hemostasis mechanism causes massive clotting in microcirculation; as clotting factors are consumed and lost, bleeding occurs; symptoms are related to tissue ischemia and bleeding

32. Disseminated Intravascular Coagulation • Manifestations include simultaneous bleeding and thrombosis; • Pallor, petechiae, purpura, oozing, bleeding, hematomas, cyanosis, tissue necrosis… • Lab tests - prolonged PT/PTT, elevated d dimer • See table 31-20 • Treatment: treat underlying cause, correct tissue ischemia, replace fluids and electrolytes, maintain blood pressure, replace coagulation factors, and use heparin

33. Heparin Induced Thrombocytopenia • Complication of heparin therapy • Higher risk with prolonged use • Hallmark sign is decline in platelet count, usually after 4-14 days (usually drops by 50% or more) • Patients at risk for bleeding; also for thrombosis (venous or arterial) • Treatment includes cessation of heparin, institution of alternative anticoagulation; follow platelet count

34. Review Heparin, Low Molecular Weight Heparin, and Warfarin therapies • Pharmacology book

35. Nursing Process—Assessment of the Patient With DIC • Be aware of patients who are at risk for DIC and assess for signs and symptoms of the condition • Assess for signs and symptoms and progression of thrombi and bleeding

36. Nursing Process—Diagnosis of the Patient With DIC • Risk for fluid volume deficiency • Risk for impaired skin integrity • Risk for imbalanced fluid volume • Ineffective tissue perfusion • Death anxiety

37. Collaborative Problems/Potential Complications • Renal failure • Gangrene • Pulmonary embolism or hemorrhage • Acute respiratory distress syndrome • Stroke

38. Nursing Process--Planning the Care of the Patient With DIC • Major goals include maintenance of hemodynamic status, maintenance of intact skin and oral mucosa, maintenance of fluid balance, maintenance of tissue perfusion, enhanced coping, and absence of complications

39. Interventions • Assessment and interventions should target potential /actual sites of organ damage • Monitor and assess carefully for bleeding and thrombosis • Avoid trauma and procedures that increase the risk of bleeding, including activities that increase intracranial pressure

40. Blood Transfusion Administration • Review patient history including history of transfusions and transfusion reactions; note concurrent health problems and obtain baseline assessment and VS • Perform patient teaching and obtain consent • Equipment: IV (19-20 gauge or greater for PRBCs), appropriate tubing, and normal saline solution • Procedure to identify patient and blood product • Monitoring of patient and VS • Postprocedure care • Nursing management of adverse reactions

41. Complicationssee table 31-33! • Febrile nonhemolytic reaction • Acute hemolytic reaction • Allergic reaction (mild or severe) • Circulatory overload • Bacterial contamination • Transfusion-related acute lung injury • Delayed hemolytic reaction • Disease acquisition • Complications of long-term transfusion therapy

42. Alternative to blood transfusion • Growth factors • Erythropoietin • Granulocyte-Colony Stimulating Factor • Granulocyte-Macrophage Colony Stimulating Factor • Thrombopoeitin

43. Peripheral Blood Stem Cell Tranplantation and Bone Marrow Tranplantation • Autologous stem cell transplantation - uncommon • Donor tranplantation - marrow stem cells or bone marrow from a donor are transfused • First, patient undergoes intense chemo or radiation to ablate the patient’s own marrow • Success depends on tissue compatibility and the patient’s tolerance of the immunosuppression • Nursing management is focused on prevention of infection and early detection of complication