Chapter 103 • 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.
Hairy Cell Leukemia
Summary of Key Points
Hairy Cell Leukemia
