Updated consensus guidelines for the diagnosis and management of patients with HCL and HCL variant
Abstract
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 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.
Article Details
Authors (47)
Clive S. Zent
1Department of Medicine and Wilmot Cancer Institute, University of Rochester Medical Center, Rochester, NY
Enrico Tiacci
University of Perugia, Perugia, Italy
Robert J. Kreitman
3Laboratory of Molecular Biology, Centers for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD
Tamar Tadmor
Bnai Zion Medical Center, Technion, Haifa, Israel
Martin S. Tallman
5Memorial Sloan Kettering Cancer Center, New York, NY
Bernhard Wörmann
6Charité–Universitätsmedizin Berlin, Berlin, Germany
Leslie A. Andritsos
7University of New Mexico Comprehensive Cancer Center, Albuquerque, NM
Evgeny Arons
3Laboratory of Molecular Biology, Centers for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD
Versha Banerji
8Paul Albrechtsen Research Institute and Department of Medical Oncology & Hematology, CancerCare Manitoba, and Department of Internal Medicine, Rady College of Medicine, University of Manitoba, Winnipeg, MB, Canada
Jacqueline C. Barrientos
9Mount Sinai Comprehensive Cancer Center, Miami Beach, FL
Seema A. Bhat
10Division of Hematology, Department of Internal Medicine, The Ohio State University, Columbus, OH
James S. Blachly
10Division of Hematology, Department of Internal Medicine, The Ohio State University, Columbus, OH
Alessandro Broccoli
Timothy G. Call
13Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN
Claire Dearden
14Royal Marsden NHS Foundation Trust, Sutton, United Kingdom
Judit Demeter
15University, Faculty of Medicine, Department of Internal Medicine and Oncology, Budapest, Hungary
Sascha Dietrich
Dima El-Sharkawi
14Royal Marsden NHS Foundation Trust, Sutton, United Kingdom
Andrei Fagarasanu
18Department of Medicine, University of Alberta, Edmonton, Alberta, Canada
Brunangelo Falini
University of Perugia, Perugia, Italy
Francesco Forconi
19Cancer Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom
Alina S. Gerrie
21BC Cancer Centre for Lymphoid Cancer, University of British Columbia, Vancouver, BC, Canada
Douglas E. Gladstone
Alessandro Gozzetti
University of Siena, Siena, Italy
Paul J. Hampel
13Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN
David J. Hermel
25Division of Hematology and Oncology, Scripps Clinic Medical Group, La Jolla, CA
Sunil Iyengar
14Royal Marsden NHS Foundation Trust, Sutton, United Kingdom
James B. Johnston
8Paul Albrechtsen Research Institute and Department of Medical Oncology & Hematology, CancerCare Manitoba, and Department of Internal Medicine, Rady College of Medicine, University of Manitoba, Winnipeg, MB, Canada
Gunnar Juliusson
Thomas J. Kipps
University of California, San Diego School of Medicine, La Jolla, California, United States
Francesco Lauria
28University of Siena, Siena, Italy
Gerard Lozanski
29Department of Pathology, The Ohio State University Medical School, Columbus, OH
Sameer A. Parikh
13Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN
Jae H. Park
Aaron Polliack
32Hadassah Hebrew University Medical Center, Jerusalem, Israel
Graeme Quest
33Department of Pathology and Molecular Medicine, Kingston Health Sciences Centre and Queen's University, Kingston, ON, Canada
Kanti Rai
34Feinstein Institutes for Medical Research, Manhasset, NY
Farhad Ravandi
Department of Leukemia The University of Texas MD Anderson Cancer Center Houston Texas USA
Tadeusz Robak
36Department of Hematology, Medical University of Lodz, Lodz, Poland
Kerry A. Rogers
10Division of Hematology, Department of Internal Medicine, The Ohio State University, Columbus, OH
Alan Saven
25Division of Hematology and Oncology, Scripps Clinic Medical Group, La Jolla, CA
John F. Seymour
Department of Haematology, Royal Melbourne Hospital and Peter MacCallum Cancer Centre, Melbourne, VIC, Australia
Constantine S. Tam
40Department of Haematology, Alfred Hospital and Monash University, Melbourne, VIC, Australia
Xavier Troussard
41Hématologie, CHU de Caen, Caen, France
Thorsten Zenz
Pier Luigi Zinzani
12IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia “Seràgnoli,” Dipartimento di Scienze Mediche e Chirurgiche, Università di Bologna, Bologna, Italy
Michael R. Grever
10Division of Hematology, Department of Internal Medicine, The Ohio State University, Columbus, OH