Hairy Cell Leukemia
Summary of Key Points
• Hairy cell leukemia (HCL) is an uncommon clonal B-cell lymphoproliferative disorder.
• BRAF V600E mutation is present in nearly all cases of HCL but absent in other B-cell lymphoproliferative disorders and represents a disease-defining genetic event in HCL.
• Physical findings generally are confined to splenomegaly.
• The purine analogs are the therapeutic agents of choice.
• Most patients who receive treatment with cladribine or pentostatin have prolonged survival.
• BL22 or HA22, an immunoconjugate of an anti-CD22 antibody linked to a truncated Pseudomonas exotoxin A, is a novel agent that has been very effective in the management of relapsed and refractory HCL.
• BRAF inhibitors represent the first molecularly targeted therapy in HCL and will soon be explored in clinical studies.
