Recent advances in diagnosis and treatment of hairy cell leukemia

Abstract

Hairy cell leukemia (HCL) is a rare, indolent B-cell lymphoproliferative disorder that accounts for 2% of all cases of leukemia. Most patients present with pancytopenia and splenomegaly with variable number of ‘hairy’ lymphocytes in blood. BRAF V600E mutation can be detected in virtually 100% of HCL cases and is absent in other B-cell lymphomas. The mutated gene and its responding abnormal protein can be used as specific markers in the diagnosis of HCL. New therapeutic modalities targeting on mutated BRAF and its downstream pathways have shown encouraging results in clinical trials. The objective of this review article is to discuss the recent developments in the diagnosis and management of hairy cell leukemia.