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Blood. 2017 Feb 2;129(5):553-560. doi: 10.1182/blood-2016-01-689422. Epub 2016 Nov 30.

Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia.

Author information

1
Division of Hematology, Department of Internal Medicine, The Ohio State University James Cancer Hospital, Columbus, OH.
2
Leukemia Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
3
Section of Hematology/Oncology, University of Manitoba, Winnipeg, MB, Canada.
4
Department of Medicine, Hofstra North Shore-Long Island Jewish School of Medicine, Hofstra University, Hempstead, NY.
5
Division of Hematology, Mayo Clinic, Rochester, MN.
6
Division of Molecular Pathology, The Institute of Cancer Research, London, United Kingdom.
7
Department of Haemato-Oncology, Royal Marsden Biomedical Research Centre, London, United Kingdom.
8
First Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
9
Haematology Department, University Hospital Trust and Cancer Sciences Unit, Cancer Research UK and National Institute for Health Research Experimental Cancer Medicine Centres, Faculty of Medicine, University of Southampton, Southampton, United Kingdom.
10
Hematology, Azienda Ospedaliera Universitaria Senese, Siena, Italy.
11
Department of Medicine V, University of Heidelberg, Heidelberg, Germany.
12
Department of Hematology, Skåne University Hospital and Stem Cell Center, Lund University, Lund, Sweden.
13
Laboratory of Molecular Biology, National Cancer Institute, National Institutes of Health, Bethesda, MD.
14
Department of Medicine, University of Alberta, Edmonton, AB, Canada.
15
Department of Pathology, The Ohio State University, Columbus, OH.
16
Department of Hematology, Hospital Clinic, University of Barcelona, Barcelona, Spain.
17
Department of Hematology, Hadassah University Hospital and Hebrew University Medical School, Jerusalem, Israel.
18
Department of Laboratory Medicine and Pathology, University Health Network, Toronto, ON, Canada.
19
Section of Developmental Therapeutics, Department of Leukemia, University of Texas MD Anderson Cancer Center, Houston, TX.
20
Department of Hematology, Medical University of Lodz, Lodz, Poland.
21
Division of Hematology and Oncology, Scripps Clinic, La Jolla, CA.
22
Haematology Department, Peter MacCallum Cancer Centre, University of Melbourne, Melbourne, VIC, Australia.
23
Hematology Unit, Bnai-Zion Medical Center, and the Rappaport Faculty of Medicine, Technion Institute of Technology, Haifa, Israel.
24
Institute of Hematology, Department of Medicine, University and Hospital of Perugia, Perugia, Italy.
25
Department of Hematology, Centre Hospitalier Universitaire Côte de Nacre, Caen, France.
26
James P. Wilmot Cancer Institute, University of Rochester Medical Center, Rochester, NY.
27
Department of Molecular Therapy in Hematology and Oncology, National Center for Tumor Diseases and German Cancer Research Center (DKFZ), Heidelberg, Germany; and.
28
Institute of Hematology "Seràgnoli," University of Bologna, Bologna, Italy.

Abstract

Hairy cell leukemia is an uncommon hematologic malignancy characterized by pancytopenia and marked susceptibility to infection. Tremendous progress in the management of patients with this disease has resulted in high response rates and improved survival, yet relapse and an appropriate approach to re-treatment present continuing areas for research. The disease and its effective treatment are associated with immunosuppression. Because more patients are being treated with alternative programs, comparison of results will require general agreement on definitions of response, relapse, and methods of determining minimal residual disease. The development of internationally accepted, reproducible criteria is of paramount importance in evaluating and comparing clinical trials to provide optimal care. Despite the success achieved in managing these patients, continued participation in available clinical trials in the first-line and particularly in the relapse setting is highly recommended. The Hairy Cell Leukemia Foundation convened an international conference to provide common definitions and structure to guide current management. There is substantial opportunity for continued research in this disease. In addition to the importance of optimizing the prevention and management of the serious risk of infection, organized evaluations of minimal residual disease and treatment at relapse offer ample opportunities for clinical research. Finally, a scholarly evaluation of quality of life in the increasing number of survivors of this now manageable chronic illness merits further study. The development of consensus guidelines for this disease offers a framework for continued enhancement of the outcome for patients.

PMID:
27903528
PMCID:
PMC5290982
DOI:
10.1182/blood-2016-01-689422
[Indexed for MEDLINE]
Free PMC Article

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