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Wierda WG , Byrd JC , Abramson JS , Bhat S , Bociek G , Brander D , Brown J , Chanan-Khan A , Coutre SE , Davis RS , Fletcher CD , Hill B , Kahl BS , Kamdar M , Kaplan LD , Khan N , Kipps TJ , Lancet J , Ma S , Malek S , Mosse C , Shadman M , Siddiqi T , Stephens D , Wagner N , Zelenetz AD , Dwyer MA , Sundar H
Hairy Cell Leukemia, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology
J Natl Compr Canc Netw. 2017 Nov;15(11) :1414-1427
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Abstract
Hairy cell leukemia (HCL) is a rare type of indolent B-cell leukemia, characterized by symptoms of fatigue and weakness, organomegaly, pancytopenia, and recurrent opportunistic infections. Classic HCL should be considered a distinct clinical entity separate from HCLvariant (HCLv), which is associated with a more aggressive disease course and may not respond to standard HCL therapies. Somatic hypermutation in the IGHV gene is present in most patients with HCL. The BRAF V600E mutation has been reported in most patients with classic HCL but not in those with other B-cell leukemias or lymphomas. Therefore, it is necessary to distinguish HCLv from classic HCL. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnosis and management of classic HCL.
Notes
Wierda, William G Byrd, John C Abramson, Jeremy S Bhat, Seema Bociek, Greg Brander, Danielle Brown, Jennifer Chanan-Khan, Asher Coutre, Steve E Davis, Randall S Fletcher, Christopher D Hill, Brian Kahl, Brad S Kamdar, Manali Kaplan, Lawrence D Khan, Nadia Kipps, Thomas J Lancet, Jeffrey Ma, Shuo Malek, Sami Mosse, Claudio Shadman, Mazyar Siddiqi, Tanya Stephens, Deborah Wagner, Nina Zelenetz, Andrew D Dwyer, Mary A Sundar, Hema eng Practice Guideline J Natl Compr Canc Netw. 2017 Nov;15(11):1414-1427. doi: 10.6004/jnccn.2017.0165.