Hairy Cell Leukemia

Pathogenesis

  • BRAF V600E Mutation

Clinical

  • Asymptomatic (25% of cases)
  • Splenomegaly
  • Pancytopenia
  • Low Monocyte Count: increased risk of infections
  • “Fried-Egg” Appearance of Cells

Treatment

  • Indications for Treatment
    • Splenomegaly
    • Significant Cytopenias
  • Agents
    • Cladribine (aka 2-Chlordeoxyadenosine) (see Cladribine, [[Cladribine]]): purine analogue
    • Pentostatin (see Pentostatin, [[Pentostatin]])
    • Rituximab (see Anti-CD20 Therapy, [[Anti-CD20 Therapy]])
    • Anti-CD22 Therapy (see Anti-CD22 Therapy, [[Anti-CD22 Therapy]])
      • CAT-3888 (aka BL22): anti-CD22 complexed with Pseudomonas exotoxin PE38
      • CAT-8015 (aka HA22, Moxetumomab Pasudotox): anti-CD22 complexed with PE38 (a 38-kDa portion of Pseudomonas exotoxin A)
    • Vemurafenib (Zelboraf) (see Vemurafenib, [[Vemurafenib]]): 1 case report from NEJM

References

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