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Hairy Cell Leukemia

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Indolent Lymphomas

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

Abstract

Hairy cell leukemia (HCL) is a rare chronic B-cell neoplasm characterized by marked splenomegaly, progressive pancytopenia, and reactive marrow fibrosis. The annual incidence of HCL is estimated to be 0.3 cases per 100,000. Diagnosis is made by a review of peripheral blood smear, flow cytometry, and bone marrow biopsy. The hairy cells have a typical pattern of B-cell antigen expression (CD19, CD20) and coexpression of CD11c, CD25, and CD103. Recently, the BRAF-V600E mutation has been described as a disease-defining genetic event. Purine nucleoside analogs, cladribine and pentostatin, are the drugs of choice in the treatment of HCL. Recently, immunotoxin moxetumomab pasudotox has been approved by FDA for the treatment of patients with relapsed or refractory HCL. Vemurafenib, which inhibits the BRAF-V600E mutant, exhibits remarkable activity in multiply relapsed and refractory patients with classic HCL.

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Authors and Affiliations

  1. Department of Hematology, Medical University of Lodz, Lodz, Poland

    Tadeusz Robak

  2. Department of Medicine V, University of Heidelberg, Heidelberg, Germany

    Sascha Dietrich

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Correspondence to Tadeusz Robak .

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  1. Department of Medicine III, LMU Hospital, München, Bayern, Germany

    Martin Dreyling

  2. Azienda Ospedaliera Nazionale SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy

    Marco Ladetto

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Robak, T., Dietrich, S. (2021). Hairy Cell Leukemia. In: Dreyling, M., Ladetto, M. (eds) Indolent Lymphomas . Hematologic Malignancies. Springer, Cham. https://doi.org/10.1007/978-3-030-55989-2_12

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