title: Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia.
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.
authors.affiliation: Division of Hematology, Department of Internal Medicine, The Ohio State University James Cancer Hospital, Columbus, OH.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Grever
authors.affiliation: Leukemia Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Omar
authors.initials: O
authors.lastname: Abdel-Wahab
authors.affiliation: Division of Hematology, Department of Internal Medicine, The Ohio State University James Cancer Hospital, Columbus, OH.
authors.forename: Leslie A
authors.initials: LA
authors.lastname: Andritsos
authors.affiliation: Section of Hematology/Oncology, University of Manitoba, Winnipeg, MB, Canada.
authors.forename: Versha
authors.initials: V
authors.lastname: Banerji
authors.affiliation: Department of Medicine, Hofstra North Shore-Long Island Jewish School of Medicine, Hofstra University, Hempstead, NY.
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Barrientos
authors.affiliation: Division of Hematology, Department of Internal Medicine, The Ohio State University James Cancer Hospital, Columbus, OH.
authors.forename: James S
authors.initials: JS
authors.lastname: Blachly
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Timothy G
authors.initials: TG
authors.lastname: Call
authors.affiliation: Division of Molecular Pathology, The Institute of Cancer Research, London, United Kingdom.
authors.forename: Daniel
authors.initials: D
authors.lastname: Catovsky
authors.affiliation: Department of Haemato-Oncology, Royal Marsden Biomedical Research Centre, London, United Kingdom.
authors.forename: Claire
authors.initials: C
authors.lastname: Dearden
authors.affiliation: First Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
authors.forename: Judit
authors.initials: J
authors.lastname: Demeter
authors.affiliation: Division of Molecular Pathology, The Institute of Cancer Research, London, United Kingdom.
authors.forename: Monica
authors.initials: M
authors.lastname: Else
authors.affiliation: 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.
authors.forename: Francesco
authors.initials: F
authors.lastname: Forconi
authors.affiliation: Hematology, Azienda Ospedaliera Universitaria Senese, Siena, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gozzetti
authors.affiliation: Department of Medicine V, University of Heidelberg, Heidelberg, Germany.
authors.forename: Anthony D
authors.initials: AD
authors.lastname: Ho
authors.affiliation: Section of Hematology/Oncology, University of Manitoba, Winnipeg, MB, Canada.
authors.forename: James B
authors.initials: JB
authors.lastname: Johnston
authors.affiliation: Division of Hematology, Department of Internal Medicine, The Ohio State University James Cancer Hospital, Columbus, OH.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Jones
authors.affiliation: Department of Hematology, Skåne University Hospital and Stem Cell Center, Lund University, Lund, Sweden.
authors.forename: Gunnar
authors.initials: G
authors.lastname: Juliusson
authors.affiliation: Division of Hematology, Department of Internal Medicine, The Ohio State University James Cancer Hospital, Columbus, OH.
authors.forename: Eric
authors.initials: E
authors.lastname: Kraut
authors.affiliation: Laboratory of Molecular Biology, National Cancer Institute, National Institutes of Health, Bethesda, MD.
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Kreitman
authors.affiliation: Department of Medicine, University of Alberta, Edmonton, AB, Canada.
authors.forename: Loree
authors.initials: L
authors.lastname: Larratt
authors.affiliation: Hematology, Azienda Ospedaliera Universitaria Senese, Siena, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Lauria
authors.affiliation: Department of Pathology, The Ohio State University, Columbus, OH.
authors.forename: Gerard
authors.initials: G
authors.lastname: Lozanski
authors.affiliation: Department of Hematology, Hospital Clinic, University of Barcelona, Barcelona, Spain.
authors.forename: Emili
authors.initials: E
authors.lastname: Montserrat
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN.
authors.forename: Sameer A
authors.initials: SA
authors.lastname: Parikh
authors.affiliation: Leukemia Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Jae H
authors.initials: JH
authors.lastname: Park
authors.affiliation: Department of Hematology, Hadassah University Hospital and Hebrew University Medical School, Jerusalem, Israel.
authors.forename: Aaron
authors.initials: A
authors.lastname: Polliack
authors.affiliation: Department of Laboratory Medicine and Pathology, University Health Network, Toronto, ON, Canada.
authors.forename: Graeme R
authors.initials: GR
authors.lastname: Quest
authors.affiliation: Department of Medicine, Hofstra North Shore-Long Island Jewish School of Medicine, Hofstra University, Hempstead, NY.
authors.forename: Kanti R
authors.initials: KR
authors.lastname: Rai
authors.affiliation: Section of Developmental Therapeutics, Department of Leukemia, University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Farhad
authors.initials: F
authors.lastname: Ravandi
authors.affiliation: Department of Hematology, Medical University of Lodz, Lodz, Poland.
authors.forename: Tadeusz
authors.initials: T
authors.lastname: Robak
authors.affiliation: Division of Hematology and Oncology, Scripps Clinic, La Jolla, CA.
authors.forename: Alan
authors.initials: A
authors.lastname: Saven
authors.affiliation: Haematology Department, Peter MacCallum Cancer Centre, University of Melbourne, Melbourne, VIC, Australia.
authors.forename: John F
authors.initials: JF
authors.lastname: Seymour
authors.affiliation: Hematology Unit, Bnai-Zion Medical Center, and the Rappaport Faculty of Medicine, Technion Institute of Technology, Haifa, Israel.
authors.forename: Tamar
authors.initials: T
authors.lastname: Tadmor
authors.affiliation: Leukemia Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Martin S
authors.initials: MS
authors.lastname: Tallman
authors.affiliation: Haematology Department, Peter MacCallum Cancer Centre, University of Melbourne, Melbourne, VIC, Australia.
authors.forename: Constantine
authors.initials: C
authors.lastname: Tam
authors.affiliation: Institute of Hematology, Department of Medicine, University and Hospital of Perugia, Perugia, Italy.
authors.forename: Enrico
authors.initials: E
authors.lastname: Tiacci
authors.affiliation: Department of Hematology, Centre Hospitalier Universitaire Côte de Nacre, Caen, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Troussard
authors.affiliation: James P. Wilmot Cancer Institute, University of Rochester Medical Center, Rochester, NY.
authors.forename: Clive S
authors.initials: CS
authors.lastname: Zent
authors.affiliation: Department of Molecular Therapy in Hematology and Oncology, National Center for Tumor Diseases and German Cancer Research Center (DKFZ), Heidelberg, Germany; and.
authors.forename: Thorsten
authors.initials: T
authors.lastname: Zenz
authors.affiliation: Institute of Hematology "Seràgnoli," University of Bologna, Bologna, Italy.
authors.forename: Pier Luigi
authors.initials: PL
authors.lastname: Zinzani
authors.affiliation: Institute of Hematology, Department of Medicine, University and Hospital of Perugia, Perugia, Italy.
authors.forename: Brunangelo
authors.initials: B
authors.lastname: Falini
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D017338:Cladribine / Q000627:therapeutic use*; D019468:Disease Management; D006801:Humans; D007943:Leukemia, Hairy Cell / Q000175:diagnosis* / Q000188:drug therapy*; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000188:drug therapy; D018365:Neoplasm, Residual / Q000175:diagnosis / Q000188:drug therapy; D015649:Pentostatin / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D015649:Pentostatin; D017338:Cladribine