Hematologic Neoplasms Notes

Management of Patients with Hematologic Neoplasms

Leukemia

  • Hematopoietic malignancy characterized by unregulated proliferation of leukocytes.
  • Types:
    • Acute myeloid leukemia (AML)
    • Chronic myeloid leukemia (CML)
    • Acute lymphocytic leukemia (ALL)
    • Chronic lymphocytic leukemia (CLL)
Acute Myeloid Leukemia (AML)
  • Defect in stem cells that differentiate into myeloid cells (monocytes, granulocytes, erythrocytes, platelets).
  • Most common nonlymphocytic leukemia.
  • Affects all ages, peak incidence at 67 years.
  • Variable prognosis.
  • Manifestations:
    • Fever and infection
    • Weakness and fatigue
    • Bleeding tendencies
    • Pain from enlarged liver or spleen
    • Hyperplasia of gums
    • Bone pain
  • Treatment:
    • Aggressive chemotherapy—induction therapy
    • Hematopoietic stem cell transplantation (HSCT)
    • Supportive care (antimicrobial therapy and transfusions)
  • Death can occur within months.
Chronic Myeloid Leukemia (CML)
  • Mutation in myeloid stem cell, leading to uncontrolled proliferation.
  • Philadelphia chromosome.
  • Stages: chronic phase, transformational phase, blast crisis.
  • Uncommon in people younger than age 20 years; increased incidence with age; mean age: 64 years.
  • Manifestations:
    • Initially asymptomatic
    • Malaise
    • Anorexia
    • Weight loss
    • Confusion or shortness of breath (leukostasis)
    • Enlarged tender spleen or liver
  • Treatment:
    • Imatinib mesylate (Gleevec) blocks signals in leukemic cells expressing BCR-ABL protein.
    • Chemotherapy
    • HSCT
Acute Lymphocytic Leukemia (ALL)
  • Uncontrolled proliferation of immature lymphoid stem cells.
  • Most common in young children; more common in boys; peak age 4 years.
  • Prognosis is good for children (85% for 3-year event-free survival), but decreases with age (<45% adults).
  • Manifestations:
    • Pain from enlarged liver/spleen, bone, CNS
    • Headache and vomiting
  • Treatment:
    • Chemotherapy
    • HSCT
    • Monoclonal antibody therapy
    • Corticosteroids
Chronic Lymphocytic Leukemia (CLL)
  • Common malignancy of older adults; most prevalent type of adult leukemia; mean age: 72 years.
  • Derived from malignant clone of B lymphocytes.
  • Survival varies from 2 to 14 years depending on stage.
  • Manifestations:
    • "B symptoms": fevers, drenching sweats (especially at night), unintentional weight loss
  • Treatment:
    • Early-stage: "watch and wait"
    • Chemotherapy
    • Monoclonal antibody therapy
    • IVIG for recurrent infections
    • HSCT

Assessment of the Patient with Leukemia

  • Health history
  • Assess symptoms:
    • Anemia
    • Infection
    • Bleeding
    • Weakness and fatigue
  • Laboratory tests:
    • Leukocyte count, ANC, hematocrit, platelets
    • Creatinine and electrolyte levels
    • Coagulation and hepatic function tests
    • Cultures as needed

Collaborative Problems and Potential Complications of Leukemia

  • Infection
  • Bleeding/DIC
  • Renal dysfunction
  • Tumor lysis syndrome

Planning and Goals for the Patient with Leukemia

  • Absence of complications and pain
  • Attainment and maintenance of adequate nutrition
  • Activity tolerance
  • Ability to provide self-care and cope with diagnosis and prognosis
  • Positive body image
  • Understanding of the disease process and its treatment

Interventions for the Patient with Leukemia

  • Risk of infection and bleeding
  • Mucositis:
    • Frequent, gentle oral hygiene
    • Soft toothbrush or sponge-tipped applicators
    • Rinse only with NS, NS and baking soda, or prescribed solutions
    • Perineal and rectal care
  • Improve nutritional intake:
    • Oral care before and after meals
    • Administer analgesics before meals
    • Appropriate treatment of nausea
    • Small, frequent feedings
    • Soft foods that are moderate in temperature
    • Low-microbial diet
    • Nutritional supplements
  • Easing pain and discomfort:
    • Acetaminophen for fever and myalgias
    • Cool water sponging
    • Frequent bedding changes
    • Gentle massage
    • Relaxation techniques
  • Decreasing fatigue and activity intolerance:
    • Balance activity and rest
  • Maintaining fluid and electrolyte balance:
    • Intake and output, daily weights
    • Assess for dehydration and overload
    • Laboratory studies (electrolytes, BUN, creatinine, hematocrit)
    • Replacement as necessary
  • Improve self-care, self-esteem, anxiety, and grief with empathetic listening and realistic reassurance

Myelodysplastic Syndromes (MDS)

  • Disorder of the myeloid stem cell.
  • May be asymptomatic or present with fatigue or illness.
  • Diagnosed with CBC or bone marrow biopsy.
  • Occurs in older adults: mean 65 to 70 years old.
  • Only cure is with HSCT.
  • Other treatments: blood transfusion, bone marrow–stimulating agents, immunosuppressive therapy, chelation therapy, and myeloid growth factors

Myeloproliferative Neoplasms

  • Polycythemia vera
  • Essential thrombocythemia
  • Primary myelofibrosis
Polycythemia Vera
  • Proliferative disorder of myeloid stem cells.
  • Median age 60; survival 14 to 20 years.
  • Symptoms: ruddy complexion, splenomegaly, high blood pressure, generalized pruritis, and erythromelalgia.
  • Diagnosis: elevated hemoglobin or hematocrit and acquired mutation in the JAK2JAK2 gene.
  • Risks: Thrombosis complications (CVA, MI) and bleeding from dysfunctional platelets.
  • Treatment:
    • Phlebotomy (initially 500 mL once or twice a week).
    • Chemotherapeutic agents to suppress marrow function.
    • Aggressive management of atherosclerosis.
    • Allopurinol or rasburicase to prevent gout.
    • Aspirin for pain.
    • Platelet aggregation inhibitors.
    • Interferon-alfa.
Essential Thrombocythemia
  • Also called primary thrombocythemia
  • Stem cell disorder within the bone marrow.
  • Cause is unknown; affects women more than men; median age 65 to 70 years.
  • Symptoms: vascular occlusion, headaches, enlarged spleen, and hemorrhage.
  • Treatment based on risk for developing thrombosis or hemorrhage and the presence of symptoms.
  • Refer to Table 30-2.
Primary Myelofibrosis
  • Chronic myeloproliferative disorder within the stem cell.
  • Disease of older adults 65 to 70 years; survival rate 2 to 14 years; more common in males.
  • Pancytopenia is common.
  • Symptoms: enlarged spleen, fatigue, pruritus, bone pain, weight loss, infection, bleeding, and cachexia.
  • Treatment based on reducing disease burden (decreasing symptoms and splenomegaly) and improving blood counts.
  • Splenectomy may be used to control significant problems.
  • Treatment:
    • Blood transfusions and erythroid stimulating agents for anemia
    • HSCT useful in younger people, only current therapy to reduce fibrosis of marrow
    • Splenectomy may be used to control significant problems

Lymphoma

  • Neoplasm of lymphoid origin.
  • Usually starts in lymph nodes but can involve lymphoid tissue in spleen, GI tract, liver, or bone marrow.
  • Classified according to degree of cell differentiation and origin of predominant malignant cell.
  • Two major categories:
    • Hodgkin lymphoma
    • Non-Hodgkin lymphoma
Hodgkin Disease
  • Relatively rare malignancy with a high cure rate.
  • Suspected viral etiology, familial pattern; incidence in early 20s and again after age 50; more common in men.
  • Unicentric; initiates in a single node.
  • Reed–Sternberg cell.
  • Manifestations: painless lymph node enlargement; pruritus; B symptoms: fever, sweats, weight loss.
  • Treatment: chemotherapy, radiation therapy, or both, and HSCT for advanced disease; determined by disease stage.
Non-Hodgkin Lymphoma (NHL)
  • Lymphoid tissues become infiltrated with malignant cells; spread is unpredictable, and localized disease is rare.
  • Increases with age; average age being 66 years.
  • Increased in autoimmune disorders, prior cancer treatment, organ transplant, viral infections, exposure to pesticides.
  • Manifestation: lymphadenopathy, B symptoms, and symptoms associated with lymphomatous masses.
  • Treatment: interferon, chemotherapy, radiation therapy, and HSCT; determined by type and stage of disease.

Multiple Myeloma

  • Malignant disease of the most mature form of B lymphocyte—the plasma cell.
  • Incidence increases with age; median 70 years old; 5-year survival rate; no cure.
  • Manifestations: bone pain (80%), mostly back and ribs; osteoporosis and fractures related to bone destruction; hypercalcemia, renal impairment and failure, anemia.
  • Treatment: HSCT, chemotherapy, corticosteroids, radiation therapy.
  • New drugs: immunomodulatory drugs, thalidomide analogs, monoclonal antibody