Please confirm topic selection

Are you sure you want to trigger topic in your Anconeus AI algorithm?

Please confirm action

You are done for today with this topic.

Would you like to start learning session with this topic items scheduled for future?

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
  • ETIOLOGY
    • 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%
  • STUDIES
    • 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
Card
1 of 0
Private Note