Updated consensus guidelines for the diagnosis and management of patients with hairy cell leukemia (HCL) and HCL-variant

Hairy cell leukemia (HCL) and hairy cell leukemia variant (HCLv) are distinct, rare, chronic splenic B cell lymphomas/leukemias that partially overlap in clinico-pathological presentation but differ in genetic basis, prognosis, and management. HCL is caused by the BRAF-V600E kinase-activating mutation in >95% of patients, usually has excellent responses to chemotherapy with purine analogs and is also amenable to BRAF inhibitor-based targeted treatments. In contrast, HCLv lacks BRAF-V600E, requires combined therapy with purine analogs plus rituximab and generally shows less-durable responses. Here, an international team of hematologists expert on these rare diseases was convened by the Hairy Cell Leukemia Foundation to update the previous guidelines (published in 2017) by providing a summary of current methods to diagnose and manage patients with HCL and HCLv, as well as a prospective on newer targeted therapies to further improve outcome.

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Publication Details

Journal
Blood
Published
2026-04-14
DOI
https://doi.org/10.1182/blood.2025032757
Primary Topic
Chronic Lymphocytic Leukemia Research
Type
article
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0.00
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article

Updated consensus guidelines for the diagnosis and management of patients with hairy cell leukemia (HCL) and HCL-variant

Farhad Ravandi, Douglas Edward Gladstone, Constantine S. Tam, Alina S. Gerrie et al.
Blood
Chronic Lymphocytic Leukemia Research
article

Updated consensus guidelines for the diagnosis and management of patients with hairy cell leukemia (HCL) and HCL-variant

Farhad Ravandi, Douglas Edward Gladstone, Constantine S. Tam, Alina S. Gerrie, Brunangelo Falini, Sameer Ashok Parikh, Gerard Lozanski, Aaron Polliack, Tamar Tadmor, Robert J. Kreitman, C. J. Dearden, Versha Banerji, Judit Demeter, Sunil Iyengar, Leslie A. Andritsos, Paul Joseph Hampel, Kerry Anne Rogers, K. R., Seema Ali Bhat, Sascha Dietrich, Clive S. Zent, Enrico Tiacci, James B. Johnston, Evgeny Arons, Jacqueline C. Barrientos, Alan Saven, Andrei Fagarasanu, Graeme Quest, Francesco Forconi, David J. Hermel, James S. Blachly, Dima El‐Sharkawi, John Francis Seymour, Alessandro Broccoli, Pier Luigi Zinzani, T J Kipps, A Gozzetti, Thorsten Zenz, Xavier Troussard, Gunnar Juliusson, Jae H Park, Francesco Lauria, Martin S. Tallman, Timothy G. Call, Tadeusz Robak, Michael Rhodes Grever, Bernhard J. Wörmann
article en

Abstract

Hairy cell leukemia (HCL) and hairy cell leukemia variant (HCLv) are distinct, rare, chronic splenic B cell lymphomas/leukemias that partially overlap in clinico-pathological presentation but differ in genetic basis, prognosis, and management. HCL is caused by the BRAF-V600E kinase-activating mutation in >95% of patients, usually has excellent responses to chemotherapy with purine analogs and is also amenable to BRAF inhibitor-based targeted treatments. In contrast, HCLv lacks BRAF-V600E, requires combined therapy with purine analogs plus rituximab and generally shows less-durable responses. Here, an international team of hematologists expert on these rare diseases was convened by the Hairy Cell Leukemia Foundation to update the previous guidelines (published in 2017) by providing a summary of current methods to diagnose and manage patients with HCL and HCLv, as well as a prospective on newer targeted therapies to further improve outcome.

Blood
Semmelweis University (HU), University of Siena (IT), Northwell Health (US), The Royal Melbourne Hospital (AU), Feinstein Institute for Medical Research (US), Mayo Clinic (US), Memorial Sloan Kettering Cancer Center (US), The University of Texas MD Anderson Cancer Center (US), University of British Columbia (CA), Institute of Cancer Research (GB), University of Alberta (CA), University of New Mexico (US), Lund University (SE), University of Zurich (CH), Queen's University (CA), University of Perugia (IT), Peter MacCallum Cancer Centre (AU), University of Rochester Medical Center (US), Jerusalem Multidisciplinary College (IL), University of California San Diego (US), CancerCare Manitoba (CA), Düsseldorf University Hospital (DE), Mount Sinai Medical Center (US), Scripps Clinic Medical Group (US), Bnai Zion Medical Center (IL), Centre Hospitalier Universitaire de Caen Normandie (FR), Azienda USL di Bologna (IT), Royal Marsden Hospital (GB), Medical University of Lodz (PL), Délégation Normandie (FR), National Cancer Institute (US), Scripps Clinic (US), University of Southampton (GB), Heinrich Heine University Düsseldorf (DE), University of Manitoba (CA), The Ohio State University (US), Monash University (AU), Charité - Universitätsmedizin Berlin (DE), Université de Caen Normandie (FR)
Openalex Percentile: Top 20%
Chronic Lymphocytic Leukemia Research
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