Hairy Cell Leukemia

Published on 04/03/2015 by admin

Filed under Hematology, Oncology and Palliative Medicine

Last modified 04/03/2015

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Chapter 103

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.

Self-Assessment Questions

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