open access

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Review article
Published online: 2021-10-05
Submitted: 2021-06-22
Accepted: 2021-08-02
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New therapeutic options for hairy cell leukemia

Anna Janowska1, Agnieszka Janus1, Tadeusz Robak12
DOI: 10.5603/AHP.a2021.0095
Affiliations
  1. Department of Hematology, Copernicus Memorial Hospital, Lodz, Poland
  2. Department of Hematology, Medical University of Lodz, Poland

open access

Ahead of print
REVIEW ARTICLE
Published online: 2021-10-05
Submitted: 2021-06-22
Accepted: 2021-08-02

Abstract

Hairy cell leukemia (HCL) is a rare B-cell lymphoproliferative disorder characterized by pancytopenia, splenomegaly and increased susceptibility to infections. In 2011, BRAF gene mutation was identified in almost all the patients with the classical type of HCL. The purine analogs cladribine and pentostatin induce long-term remission in the majority of patients, and they remain the standard treatment for this type of leukemia.

However, more than half of patients in complete response relapse over the long term, with a quarter of them relapsing within the first five years.

Recently, new drugs have been developed and have demonstrated efficacy in refractory or relapsed HCL. The immunotoxin Moxetumomab pasudotox was registered for HCL in 2018. The BRAF kinase inhibitors vemurafenib and dabrafenib, as well as the Bruton kinase inhibitor ibrutinib, are also proven highly effective in clinical trials.

Abstract

Hairy cell leukemia (HCL) is a rare B-cell lymphoproliferative disorder characterized by pancytopenia, splenomegaly and increased susceptibility to infections. In 2011, BRAF gene mutation was identified in almost all the patients with the classical type of HCL. The purine analogs cladribine and pentostatin induce long-term remission in the majority of patients, and they remain the standard treatment for this type of leukemia.

However, more than half of patients in complete response relapse over the long term, with a quarter of them relapsing within the first five years.

Recently, new drugs have been developed and have demonstrated efficacy in refractory or relapsed HCL. The immunotoxin Moxetumomab pasudotox was registered for HCL in 2018. The BRAF kinase inhibitors vemurafenib and dabrafenib, as well as the Bruton kinase inhibitor ibrutinib, are also proven highly effective in clinical trials.

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Keywords

BRAF, dabrafenib, hairy cell leukemia, ibrutinib, moxetumomab pasudotox, purine analogs, rituximab, vemurafenib

About this article
Title

New therapeutic options for hairy cell leukemia

Journal

Acta Haematologica Polonica

Issue

Ahead of print

Article type

Review article

Published online

2021-10-05

DOI

10.5603/AHP.a2021.0095

Keywords

BRAF
dabrafenib
hairy cell leukemia
ibrutinib
moxetumomab pasudotox
purine analogs
rituximab
vemurafenib

Authors

Anna Janowska
Agnieszka Janus
Tadeusz Robak

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