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Published in: Current Oncology Reports 5/2020

01-05-2020 | Rituximab | Lymphomas (MR Smith, Section Editor)

Hairy Cell Leukaemia

Authors: Matthew Cross, Claire Dearden

Published in: Current Oncology Reports | Issue 5/2020

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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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Metadata
Title
Hairy Cell Leukaemia
Authors
Matthew Cross
Claire Dearden
Publication date
01-05-2020
Publisher
Springer US
Published in
Current Oncology Reports / Issue 5/2020
Print ISSN: 1523-3790
Electronic ISSN: 1534-6269
DOI
https://doi.org/10.1007/s11912-020-00911-0

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