Hairy Cell Leukemia

Aka: Hairy Cell Leukemia, Leukemic Reticuloendotheliosis, Hairy-Cell Leukemia

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II. Epidemiology

  1. Affects patients over age 40 years
  2. More common in Males

III. Pathophysiology

  1. B Lymphocyte proliferation
  2. Commonly associated effects
    1. Splenomegaly
    2. Impaired host defense and secondary infection
    3. Vasculitis

IV. Symptoms

  1. Bone pain (e.g. Hip Pain) if bone lesions present

V. Signs

  1. Defining features
    1. Splenomegaly (75%)
  2. Vasculitis associated findings
    1. Erythema Nodosum
    2. Cutaneous Nodules
  3. Rare findings
    1. Lymphadenopathy
    2. Hepatomegaly

VI. 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

VII. Differential Diagnosis

  1. Chronic Lymphocytic Leukemia
  2. Waldenstrom's Macroglobulinemia
  3. Acute Leukemia
  4. Aplastic Anemia

VIII. 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

IX. Management: Leukemia progression

  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

X. Prevention

  1. Pneumococcal Vaccine

XI. Prognosis

  1. Eight year survival >50%

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