Abstract
Hairy cell leukemia (HCL) is a rare chronic B-cell neoplasm characterized by marked splenomegaly, progressive pancytopenia, and reactive marrow fibrosis. The annual incidence of HCL is estimated to be 0.3 cases per 100,000. Diagnosis is made by a review of peripheral blood smear, flow cytometry, and bone marrow biopsy. The hairy cells have a typical pattern of B-cell antigen expression (CD19, CD20) and coexpression of CD11c, CD25, and CD103. Recently, the BRAF-V600E mutation has been described as a disease-defining genetic event. Purine nucleoside analogs, cladribine and pentostatin, are the drugs of choice in the treatment of HCL. Recently, immunotoxin moxetumomab pasudotox has been approved by FDA for the treatment of patients with relapsed or refractory HCL. Vemurafenib, which inhibits the BRAF-V600E mutant, exhibits remarkable activity in multiply relapsed and refractory patients with classic HCL.KeywordsBRAFCladribineDabrafenibIbrutinibImmunotoxinsHairy cell leukemiaHCL variantMoxetumomab pasudotoxPentostatinRituximabVemurafenib
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