Hematology and Oncology Book

Approach

Bone Marrow Disorders

Lymphatic Disorders

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Chronic Lymphocytic LeukemiaAka: CLL

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  1. Epidemiology
    1. Most common Leukemia in the United States
    2. Elderly patients (usually over age 50 years)
    3. More common in men
    4. Rare in Asian patients
  2. Pathophysiology
    1. Neoplastic accumulation of mature Lymphocytes
      1. Involves Blood and Bone Marrow
      2. May infiltrate spleen and lymph nodes
    2. Clonal B Lymphocyte mass involved in 95% of cases
    3. Chromosomal Abnormality: Trisomy 12
  3. Symptoms
    1. Weakness
    2. Fatigue
    3. Anorexia
    4. Pruritus
    5. Frequent infections
  4. Signs
    1. Hepatomegaly
    2. Splenomegaly
    3. Lymphadenopathy
    4. Fever
  5. Labs
    1. Peripheral Smear
      1. Leukocytosis
      2. Morphologically normal small Leukocytes
      3. HemeoncCllBlood.jpg
    2. Complete Blood Count
      1. Leukocyte count 15,000 to 200,000 (80-90% mature)
      2. Decreased Platelet Count
      3. Hemoglobin or Hematocrit consistent with Anemia
  6. Differential Diagnosis
    1. B-Cell CLL
      1. Reactive Lymphocytosis
    2. T-Cell CLL
      1. Sezary Syndrome
      2. Adult T-Cell Leukemia
    3. General
      1. Prolymphocytic Leukemia
      2. LymphoSarcoma Cell Leukemia
      3. Hairy Cell Leukemia
      4. Waldenstrom's Macroglobulinemia
  7. Complications
    1. Pancytopenia
    2. Coombs-positive Hemolytic Anemia (20%)
    3. Hypogammaglobulinemia
    4. Opportunistic infection
    5. Richter's Syndrome (evolves into aggressive Lymphoma)
    6. Autoimmune Thrombocytopenia
  8. Imaging
    1. Chest XRay
      1. Enlarged mediastinal lymph nodes
  9. Management
    1. Efficacy of treatment
      1. No regimen effective at eradicating CLL
      2. Goal is to reduce Leukemia cell mass and symptoms
    2. Alkylating Agents (pulsed q3-6 weeks or continuous)
      1. Indications
        1. Hemolytic Anemia and other cytopenia
        2. Disfiguring Lymphadenopathy
        3. Symptomatic organomegaly
        4. Marked systemic symptoms
      2. Agents
        1. Chlorambucil
        2. Cyclophosphamide
    3. Glucocorticoids
      1. Coombs-positive Hemolytic Anemia
      2. Immune Thrombocytopenia
      3. Pancytopenia
      4. "Packed Marrow" Syndrome
    4. Splenectomy indications
      1. Hypersplenism
      2. Refractory Hemolytic Anemia
      3. Thrombocytopenia
    5. Radiation therapy indications
      1. Localized disease
      2. Palliative end-stage disease therapy (total-body)
    6. Immunoglobulin transfusion (not proven efficacious)
  10. Course
    1. Indolent nature
    2. Often incidental finding on Complete Blood Count
  11. Staging and Prognosis
    1. Stage A: Lymphocytosis (<3 lymph node groups involved)
      1. Median survival: over 10 years
    2. Stage B: Lymphocytosis (>3 lymph node groups involved)
      1. Median survival: 5 years
    3. Stage C: Lymphocytosis with Anemia or Thrombocytopenia
      1. Median survival: 2 years

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