leukemia, and lymphoma 

Leukemia

Definition of Leukemia

  • A group of malignant disorders that affect the blood and blood-forming tissues, notably:
      - Bone marrow
      - Lymphatic system

  • Occurs across all age groups.

  • Characterized by the accumulation of dysfunctional cells due to a loss of regulation in cell division.

  • Fatal if untreated.

  • Common misconception: Often thought of as a childhood disease.

Classification of Leukemia

  • Acute vs. Chronic
      - Acute: Characterized by clonal proliferation of immature hematopoietic cells.
      - Chronic: Involves mature forms of WBC, with more gradual onset.

Cell Line Affected

  • Acute Lymphocytic Leukemia (ALL)

  • Acute Myelogenous Leukemia (AML)

  • Chronic Myelogenous Leukemia (CML)

  • Chronic Lymphocytic Leukemia (CLL)

Etiology and Pathophysiology

  • No single causative agent identified.

  • Both genetic and environmental influences play a role in development.

  • Associated risk factors include:
      - Chemical agents
      - Chemotherapeutic agents
      - Viruses
      - Radiation
      - Immunologic deficiencies

Types of Leukemia

Table 31-25: Types of Leukemia

Type of Leukemia

Age of Onset

Clinical Manifestations

Diagnostic Findings

Acute Myelogenous Leukemia (AML)

Increase in incidence with advancing age, peak between 60-70 years

Fatigue, weakness, headache, mouth sores, anemia, bleeding, fever, infections, sternal tenderness, gingival hyperplasia, minimal hepatosplenomegaly, and lymphadenopathy.

Low RBC, Hb, Hct; low platelet; variable WBC with myeloblasts; high LDH; greatly hypercellular bone marrow with myeloblasts.

Acute Lymphocytic Leukemia (ALL)

Before 14 years, peak incidence between 2-9 and in older adults

Fever, pallor, bleeding, anorexia, fatigue, bone pain, lymphadenopathy, infections, weight loss; CNS involvement may lead to nausea, vomiting, and lethargy.

Low RBC, Hb, Hct; variable platelet count; variable WBC count; high LDH; hypercellular bone marrow with lymphoblasts; Philadelphia chromosome (20%-25% of patients) in some cases.

Chronic Myelogenous Leukemia (CML)

25-60 years, peak incidence around 45 years

Early detection may show no symptoms; fatigue, fever, weight loss, sternal tenderness, and joint pain can develop; massive splenomegaly may occur.

Low RBC, Hb, Hct; high platelet count initially, decreasing later; increase in polymorphonuclear neutrophils; normal lymphocytes; low leukocyte alkaline phosphatase; presence of Philadelphia chromosome (90% of patients).

Chronic Lymphocytic Leukemia (CLL)

50-70 years, rare below 30 years, predominance in men

Often asymptomatic; may detect during examinations for unrelated conditions; chronic fatigue, anorexia, splenomegaly, and lymphadenopathy; advanced cases may show fever.

Mild anemia, thrombocytopenia; total WBC count >100,000/µL; increased peripheral lymphocytes; hypogammaglobulinemia; potential autoimmune factors may be present.

Symptoms of Leukemia

  • Anemia: Decreased hemoglobin (Hgb) levels.

  • Neutropenia: Increased risk of infection.

  • Thrombocytopenia: Increased bleeding risk.

  • Immature White Blood Cells: Leukemias involve numerous immature WBCs, likened to "ants in an ant colony."

Pathophysiology of Leukemia

  • Proliferation of immature white blood cells: These malignant cells, or blast cells, proliferate.

  • Bone marrow failure: Blast cells replace normal bone marrow cells.

  • Cell infiltration: Cells may infiltrate sites outside the bone marrow; common sites include:
      - Erythrocytes
      - Lymphocytes
      - Platelets
      - Central Nervous System
      - Testicles

  • Symptoms may include anemia, immunosuppression, decreased clotting, weakness, pallor, infection, thrombocytopenia, fever, petechiae, bruising, purpura, along with organ infiltration into lymph nodes, liver, spleen, and joints.

Treatment of Leukemia

Anticancer Drugs

  • Adverse Reactions/Precautions:
      - Nausea and vomiting
      - Bone marrow suppression
      - Alopecia (hair loss)
      - Anorexia
      - Gastrointestinal disturbances
      - Pregnancy should be avoided during treatment.

Treatment Regimens
Acute Myelogenous Leukemia (AML)

  • Common drugs used:
      - Cytarabine (Cytosar, Ara-C)
      - Daunorubicin (Cerubidine)
      - Idarubicin (Idamycin)
      - 6-thioguanine (6-TG)
      - Mitoxantrone (Novantrone)
      - Arsenic trioxide (Trisenox)
      - Tretinoin (Vesanoid)
      - Etoposide (VePesid)
      - Clofarabine (Clolar)
      - Decitabine (Dacogen)

  • Combination chemotherapy typically includes cytarabine with an antitumor antibiotic.

Acute Lymphocytic Leukemia (ALL)

  • Common drugs used:
      - Daunorubicin
      - Doxorubicin (Adriamycin)
      - Vincristine (Oncovin)
      - Prednisone
      - Dexamethasone (Decadron)
      - L-asparaginase (Elspar)
      - Pegaspargase (Oncaspar)
      - Dasatinib (Sprycel)
      - Cyclophosphamide (Cytoxan)
      - Methotrexate
      - 6-mercaptopurine (Purinethol)
      - Nelarabine (Arranon)
      - Imatinib (Gleevec)
      - Clofarabine

  • Combination chemotherapy of several agents is common.

Chronic Myelogenous Leukemia (CML)

  • Common drugs used:
      - Imatinib
      - Dasatinib
      - Nilotinib (Tasigna)
      - Hydroxyurea (Hydrea)

  • Combination chemotherapy may include any of the following:
      - Cytarabine
      - Thioguanine
      - Daunorubicin
      - Methotrexate
      - Prednisone
      - Vincristine
      - L-asparaginase
      - Carmustine (BCNU)
      - 6-mercaptopurine

Chronic Lymphocytic Leukemia (CLL)

  • Common drugs used:
      - Chlorambucil (Leukeran)
      - Cyclophosphamide
      - Prednisone
      - Vincristine
      - Fludarabine (Fludara)
      - Rituximab (Rituxan)
      - Alemtuzumab (Campath)
      - Pentostatin (Nipent)
      - Bendamustine (Treanda)
      - Ofatumumab (Arzerra)

Other Therapies

  • Autologous or allogeneic hematopoietic stem cell transplant.

  • Cranial radiation therapy, intrathecal methotrexate or cytarabine.

  • Additional treatments may include radiation, leukapheresis, and colony-stimulating factors.

Chemotherapy

  • Goals: Achieve a remission, which can be complete, partial, or molecular.

  • Prognosis: Directly related to the ability to maintain remission.

  • Stages of therapy:
      - Induction
      - Intensification
      - Consolidation
      - Maintenance chemotherapy

Stem Cell Transplant

  • Goal: Completely eliminate leukemic cells using combinations of chemotherapy with or without total body irradiation.

  • The patient’s hematopoietic stem cells are eradicated and replaced with:
      - Stem cells from an HLA-matched sibling
      - Volunteer
      - Identical twin
      - Or patient's own stem cells as an alternative.

Lymphoma

Definition of Lymphoma

  • Malignant neoplasms originating in the bone marrow and lymphatic structures.

  • Results in the proliferation of lymphocytes.

  • Ranked as the fifth most common type of cancer in the United States.

  • Types of Lymphoma:
      - Hodgkin’s lymphoma
      - Non-Hodgkin’s lymphoma (NHL)

Hodgkin's Lymphoma

  • Characteristics:
      - Painless enlargement of lymph nodes, with progression potentially leading to liver and spleen involvement.
      - Common metastasis sites: Spleen, liver, bone marrow, and lungs.
      - The disease spreads via extension along the lymphatic system.
      - Considered the most curable lymphoma.

Risk Factors/Etiology

  1. Familial tendency, more prevalent in men.

  2. Possible causes include Epstein-Barr virus or exposure to occupational toxins.

  3. Increased incidence seen in immunosuppressed patients (e.g., HIV infection).

Clinical Manifestations, Diagnosis, and Treatment
Clinical Manifestations

  • Painless enlargement of lymph nodes (cervical, axillary, inguinal, mediastinal).

  • Symptoms often due to pressure on adjacent organs.

  • Additional symptoms:
      - Fever
      - Malaise
      - Night sweats
      - Weight loss
      - Notably, weight loss, fever, and night sweats are predictive of poor prognosis.
      - Pain in affected lymph nodes can occur upon ingestion of small amounts of alcohol.

Diagnosis

  • Lymph node biopsy: Specimen reveals presence of Reed-Sternberg cells.

Treatment

  • Typically involves chemotherapy and radiation.

Non-Hodgkin's Lymphoma

  • A neoplastic growth derived from B and T cells found within lymphoid tissue.

  • Spreads unpredictably with malignant cells infiltrating lymphoid tissue.

Risk Factors/Etiology

  1. Increased incidence in individuals with immunodeficiency or autoimmune conditions who are on immunosuppressant medications.

  2. Associated with viral infections (Epstein-Barr, hepatitis B and C).

  3. Linked to occupational exposure to carcinogens and chemicals (e.g., pesticides, herbicides, solvents).

Clinical Manifestations, Diagnosis, and Treatment
Clinical Manifestations

  • Symptoms are variable and may include lymphadenopathy that waxes and wanes.

Diagnosis

  • Confirmed through lymph node biopsy.

Treatment

  • Typically involves chemotherapy and radiation.

References

  • Huether, S.E., McCance, K.L., Brashers, V.L., & Rote, N.S. (2020). Understanding Pathophysiology (7th ed.). St. Louis: Mosby.

  • Lewis, S.L., Bucher, L., Heitkemper, M.M., Harding, M.M., Kwong, J. & Roberts, D. (2020). Medical-Surgical Nursing: Assessment and Management of Clinical Problems (11th ed.). St. Louis: Mosby.

  • Lilley, L.L., Rainforth Collins, S., Harrington, S., & Snyder, J.S. (2020). Pharmacology and the Nursing Process (9th ed.). St. Louis: Mosby.

  • Zerwekh, J., & Claborn, J.C. (2019). Illustrated Study Guide for the NCLEX-RN® Exam (10th ed.). St. Louis: Mosby.