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Hairy Cell Leukaemia

  • Lymphomas (MR Smith, Section Editor)
  • Published:
Current Oncology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

To summarise diagnostic clinical/laboratory findings and highlight differences between classical hairy cell leukaemia (HCLc) and hairy cell leukaemia variant (HCLv). Discussion of prognosis and current treatment indications including novel therapies, linked to understanding of the underlying molecular pathogenesis.

Recent Findings

Improved understanding of the underlying pathogenesis of HCLc, particularly the causative mutation BRAF V600E, leading to constitutive activation of the MEK/ERK signalling pathway and increased cell proliferation.

Summary

HCLc is caused by BRAF V600E mutation in most cases. Purine nucleoside analogue (PNA) therapy is the mainstay of treatment, with the addition of rituximab, improving response and minimal residual disease (MRD) clearance. Despite excellent responses to PNAs, many patients will eventually relapse, requiring further therapy. Rarely, patients are refractory to PNA therapy. In relapsed/refractory patients, novel targeted therapies include BRAF inhibitors (BRAFi), anti-CD22 immunoconjugate moxetumomab and Bruton tyrosine kinase inhibitors (BTKi). HCLv has a worse prognosis with median overall survival (OS), only 7–9 years, despite the combination of PNA/rituximab improving front-line response. Moxetumomab or ibrutinib may be a viable treatment but lacks substantial evidence.

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Funding

The research work (including research degree studies) of Dr. Cross is supported by funding from the Royal Marsden Cancer Charity.

Author information

Authors and Affiliations

  1. The Royal Marsden Hospital and the Institute of Cancer Research, Sutton, UK

    Matthew Cross & Claire Dearden

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  1. Matthew Cross
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  2. Claire Dearden
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Correspondence to Claire Dearden.

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Matthew Cross declares that he has no conflict of interest.

Claire Dearden has received compensation for participation on advisory boards from Innate Pharma, Roche, Janssen and AbbVie.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Lymphomas

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Cross, M., Dearden, C. Hairy Cell Leukaemia. Curr Oncol Rep 22, 42 (2020). https://doi.org/10.1007/s11912-020-00911-0

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  • DOI: https://doi.org/10.1007/s11912-020-00911-0

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