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HAIRY CELL LEUKEMIA
  • Not common
  • Less than 500 cases per year in the USA
  • Common in males
  • Affect middle-age persons

SIGNS AND SYMPTOMS

  • Generalized weakness, secondary to anemia
  • Organomegaly - enlarged spleen and liver
  • Lymph nodes may be enlarged in some cases
  • Decreased white cells leading to increased susceptibility to infections
  • Decreased monocytes
  • Increased predisposition to atypical mycobacterial infections
  • Low platelets with bleeding tendencies
  • Bone marrow aspirate is usually "dry tap"; there is increased reticulum formation
  • Bone marrow is positive for 'TRAP' (Tartrate Resistant Acid Phosphatase) test

LABORATORY TESTS

  • Positive CD19, CD20, CD11c, CD22, CD103, CD25 and surface immunoglobulin
  • Majority of patients die of infection

TREATMENT

  • 2-chlosodeoxyadenosine (2 CDA) - first line agent
  • Other treatments include deoxycoformycin (DCF), Interferon, Splenectomy
  • Life span is shortened
  • Median survival is about four years