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Updated: Dec 27 2021

Hairy Cell Leukemia (HCL)

  • Snapshot
    • A 53-year-old man presents to urgent care with a sinus infection. It’s his third infection of the month. He has been feeling very fatigued lately, but attributed that to his multiple infections. Blood-work is obtained, showing leukopenia, along with anemia and thrombocytopenia. Physical exam reveals marked splenomegaly. A peripheral blood smear shows cells with hairy cytoplasmic projections. It stains with TRAP.
  • Introduction
    • Indolent leukemia of mature B-cells with infiltration of bone marrow and spleen
    • Characteristics
      • “hairy cells” with filamentous, hair-like projections
      • tartrate-resistant acid phosphatase (TRAP +)
  • Epidemiology
    • Median onset 50-55 years of age
    • Male > female 4.5x
    • Associated conditions
      • autoimmune conditions
      • BRAF V600E mutation
  • Presentation
    • Symptoms
      • pancytopenia from infiltration of bone marrow
        • fatigue
        • infections
        • bleeding
        • anemia
    • Physical exam
      • splenomegaly from infiltration of spleen
      • may see hepatomegaly
      • may see lymphadenopathy in 10%
    • Best initial test - peripheral blood smear
      • B-cells with hairy projections
      • + TRAP stain
    • Most accurate test – flow cytometry
      • CD11c+ and CD2+
      • CD5- and CD10-
    • Bone marrow aspirate
      • dry tap due to marrow fibrosis
      • increased reticulin
    • CBC
      • anemia
      • thrombocytopenia
      • leukopenia
  • Differential Diagnosis
    • Non-Hodgkin lymphoma
    • Mantle cell lymphoma
  • Treatment
    • If asymptomatic, just observe
    • If symptomatic, treat
      • first-line is purine analogs
        • cladribine (2-CDA)
        • pentostatin
        • both inhibit adenosine deaminase
        • recall this is the same enzyme deficiency seen in SCID leading to B-cell dysfunction
      • if refractory, INFα or rituximab
  • Complications
    • Infections
    • Increased risk of malignancy
  • Prognosis
    • Good prognosis, especially with good response to purine analogs
    • Disease progression is slow
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