Leukemia

Hairy Cell Leukemia

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Hairy Cell Leukemia, Leukemic Reticuloendotheliosis, Hairy-Cell Leukemia

  • See Also
  1. Leukemia
  2. Acute Lymphocytic Leukemia (ALL)
  3. Chronic Lymphocytic Leukemia (CLL)
  • Epidemiology
  1. Affects patients over age 40 years
  2. More common in Males
  • Pathophysiology
  1. B Lymphocyte proliferation
  2. Commonly associated effects
    1. Splenomegaly
    2. Impaired host defense and secondary infection
    3. Vasculitis
  • Symptoms
  1. Bone pain (e.g. Hip Pain) if bone lesions present
  • Signs
  1. Defining features
    1. Splenomegaly (75%)
  2. Vasculitis associated findings
    1. Erythema Nodosum
    2. Cutaneous Nodules
  3. Rare findings
    1. Lymphadenopathy
    2. Hepatomegaly
  • Labs
  1. Complete Blood Count
    1. Pancytopenia
    2. Leukocyte count normal or low
  2. Peripheral Smear
    1. Hairy appearing Leukocytes (cytoplasmic projections)
      1. Size: 15 to 20 um in diameter
      2. Eccentric nucleus with foamy cytoplasm
    2. Tartrate-resistant Acid Phosphatase (TRAP) staining
  3. Bone Marrow Aspiration
    1. Difficult due to reticulin fibrosis
    2. Mononuclear cells replace normal architecture
  • Differential Diagnosis
  1. Chronic Lymphocytic Leukemia
  2. Waldenstrom's Macroglobulinemia
  3. Acute Leukemia
  4. Aplastic Anemia
  • Complications
  1. Infection (most common cause of death)
    1. Legionella pneumonitis
    2. Toxoplasmosis
    3. Mycobacterium tuberculosis
    4. Atypical Mycobacterium Disease
    5. Nocardiosis
    6. Pyogenic infection
  1. Indications
    1. Pancytopenia
    2. Infections
    3. Massive Splenomegaly
    4. Rapid disease progression
  2. Management Options
    1. Splenectomy
    2. alpha Interferon
      1. Highly effective in 70% of cases
      2. Remission is rare
    3. Pentostatin
      1. Very active in Hairy Cell Leukemia
    4. Glucocorticosteroid Indications
      1. Vasculitis
      2. Autoimmune complications
    5. Chemotherapy with Alkylating Agents contraindicated
  • Prevention
  1. Pneumococcal Vaccine
  • Prognosis
  1. Eight year survival >50%