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Hairy Cell Leukemia

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Neoplastic Diseases of the Blood

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Abstract

The WHO categorizes hairy cell leukemia (HCL) as a mature B-cell neoplasm. HCL is characterized by lymphocytes with prominent cytoplasmic projections (hairy cells) infiltrating the bone marrow and spleen, leading to pancytopenia, bone marrow fibrosis, and splenic enlargement. Hairy cells have a unique immunophenotypic profile—CD11c+, CD20+, CD25+, and CD103+—that confirms its diagnosis. The course of HCL is usually chronic, but can often be progressive, and most patients require treatment at some point. The purine nucleoside analogues, pentostatin and cladribine, are highly active, but cladribine is the preferred first-line choice due to its efficacy, brief treatment duration, and favorable toxicity profile. Other therapeutic options include rituximab, interferon-alpha, and splenectomy. With current therapy, an overall survival of 87 % at 12 years has been reported.

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Acknowledgments

The authors thank and acknowledge the gracious help offered by Dr. Richard Scuderi in compiling the pathology figures.

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Authors and Affiliations

  1. Division of Hematology/Oncology, Scripps Clinic, 10666 North Torrey Pines Road, La Jolla, CA, 92037, USA

    Sejal A. Jhatakia M.D., Darren S. Sigal M.D. & Alan Saven M.D.

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  1. Sejal A. Jhatakia M.D.
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  2. Darren S. Sigal M.D.
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  3. Alan Saven M.D.
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Correspondence to Alan Saven M.D. .

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Editors and Affiliations

  1. Beth Israel Hospital, Cancer Center, St. Lukes-Roosevelt Hospital Center, 10th Avenue 1000, New York, 10019, New York, USA

    Peter H. Wiernik

  2. , Department of Hematology, Imperial College of London, Du Cane Road 150, London, W12 0NN, United Kingdom

    John M. Goldman

  3. Continuum Cancer Centers, Department of Medicine, St. Luke's-Roosevelt Hospital Center, 10th Avenue 1000, New York, 10019, New York, USA

    Janice P. Dutcher

  4. , Division of Hematology, Mayo Clinic, First Street SW. 200, Rochester, 55905, Minnesota, USA

    Robert A. Kyle

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Jhatakia, S.A., Sigal, D.S., Saven, A. (2013). Hairy Cell Leukemia. In: Wiernik, P., Goldman, J., Dutcher, J., Kyle, R. (eds) Neoplastic Diseases of the Blood. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-3764-2_10

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