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Updated consensus guidelines for the diagnosis and management of patients with HCL and HCL variant

Zent, Clive S. ; Tiacci, Enrico ; Kreitman, Robert J. ; Tadmor, Tamar ; Tallman, Martin S. ; Wörmann, Bernhard ; Andritsos, Leslie A. ; Arons, Evgeny ; Banerji, Versha and Barrientos, Jacqueline C. , et al. (2026) In Blood
Abstract

Hairy cell leukemia (HCL) and HCL variant (HCLv) are distinct, rare, and chronic splenic B-cell lymphomas/leukemias that partially overlap in clinicopathologic presentation but differ in genetic basis, prognosis, and management. HCL is caused by the BRAF-V600E kinase–activating mutation in >95% of the patients, usually has excellent responses to chemotherapy with purine analogues, and is also amenable to BRAF inhibitor–based targeted treatments. In contrast, HCLv lacks BRAFV600E mutation, requires combined therapy with purine analogues in addition to rituximab, and generally shows less durable responses. Here, an international team of hematologists, experts on these rare diseases, was convened by the Hairy Cell Leukemia Foundation to... (More)

Hairy cell leukemia (HCL) and HCL variant (HCLv) are distinct, rare, and chronic splenic B-cell lymphomas/leukemias that partially overlap in clinicopathologic presentation but differ in genetic basis, prognosis, and management. HCL is caused by the BRAF-V600E kinase–activating mutation in >95% of the patients, usually has excellent responses to chemotherapy with purine analogues, and is also amenable to BRAF inhibitor–based targeted treatments. In contrast, HCLv lacks BRAFV600E mutation, requires combined therapy with purine analogues in addition to rituximab, and generally shows less durable responses. Here, an international team of hematologists, experts 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 outcomes.

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organization
publishing date
type
Contribution to journal
publication status
in press
subject
in
Blood
publisher
American Society of Hematology
external identifiers
  • scopus:105040723934
  • pmid:41980020
ISSN
0006-4971
DOI
10.1182/blood.2025032757
language
English
LU publication?
yes
id
e6224509-dcf5-4eb3-9151-3c25d3007777
date added to LUP
2026-09-24 15:18:10
date last changed
2026-09-24 15:18:49
@misc{e6224509-dcf5-4eb3-9151-3c25d3007777,
  abstract     = {{<p>Hairy cell leukemia (HCL) and HCL variant (HCLv) are distinct, rare, and chronic splenic B-cell lymphomas/leukemias that partially overlap in clinicopathologic presentation but differ in genetic basis, prognosis, and management. HCL is caused by the BRAF-V600E kinase–activating mutation in &gt;95% of the patients, usually has excellent responses to chemotherapy with purine analogues, and is also amenable to BRAF inhibitor–based targeted treatments. In contrast, HCLv lacks BRAFV600E mutation, requires combined therapy with purine analogues in addition to rituximab, and generally shows less durable responses. Here, an international team of hematologists, experts 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 outcomes.</p>}},
  author       = {{Zent, Clive S. and Tiacci, Enrico and Kreitman, Robert J. and Tadmor, Tamar and Tallman, Martin S. and Wörmann, Bernhard and Andritsos, Leslie A. and Arons, Evgeny and Banerji, Versha and Barrientos, Jacqueline C. and Bhat, Seema A. and Blachly, James S. and Broccoli, Alessandro and Call, Timothy G. and Dearden, Claire and Demeter, Judit and Dietrich, Sascha and El-Sharkawi, Dima and Fagarasanu, Andrei and Falini, Brunangelo and Forconi, Francesco and Gerrie, Alina S. and Gladstone, Douglas E. and Gozzetti, Alessandro and Hampel, Paul J. and Hermel, David J. and Iyengar, Sunil and Johnston, James B. and Juliusson, Gunnar and Kipps, Thomas J. and Lauria, Francesco and Lozanski, Gerard and Parikh, Sameer A. and Park, Jae H. and Polliack, Aaron and Quest, Graeme and Rai, Kanti and Ravandi, Farhad and Robak, Tadeusz and Rogers, Kerry A. and Saven, Alan and Seymour, John F. and Tam, Constantine S. and Troussard, Xavier and Zenz, Thorsten and Zinzani, Pier Luigi and Grever, Michael R.}},
  issn         = {{0006-4971}},
  language     = {{eng}},
  publisher    = {{American Society of Hematology}},
  series       = {{Blood}},
  title        = {{Updated consensus guidelines for the diagnosis and management of patients with HCL and HCL variant}},
  url          = {{http://dx.doi.org/10.1182/blood.2025032757}},
  doi          = {{10.1182/blood.2025032757}},
  year         = {{2026}},
}