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Hairy Cell Leukemia (HCL) and HCL Variant: Updates and Spotlights on Therapeutic Advances

  • Lymphomas (T Hilal, Section Editor)
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Abstract

Purpose of Review

This article aims to bring an update on the recent discoveries in hairy cell leukemia (HCL), especially findings in pathophysiology and therapeutic advances.

Recent Findings

Major discoveries have been made in genetics and epigenetics of HCL. Moreover, the importance of several signaling pathways and tumor microenvironment has been recently highlighted. These findings led to the development of new targeted therapies which have shown interesting results in recent clinical trials.

Summary

HCL is a chronic B-cell lymphoproliferative disorder. Most patients respond to purine nucleoside analogs (PNA) like cladribine or pentostatin. However, relapses are frequent and the disease often becomes less sensitive to chemotherapy. Recent discoveries in pathophysiology, like the presence of the V600E mutation of the B-raf proto-oncogene (BRAF) gene and the importance of the B-cell receptor (BCR) pathway, led to the development of new drugs for relapsed/refractory (R/R) HCL patients. The variant-type of HCL (HCL-V) is usually less sensitive to PNA. Chemo-immunotherapy using PNA and rituximab (R), BRAF, MEK, or Bruton Tyrosine Kinase (BTK) inhibitors may be used. Good results were recently published and achieved with moxetumomab pasudotox (Moxe), an anti-CD22 immunoconjugate. In this review, we will present an update on HCL and HCL-V, focusing on pathophysiology and recent therapeutic advances.

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Data Availability

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  1. Service Des Maladies du Sang, CHU d’Angers, Angers, France

    Jérôme Paillassa

  2. Hématologie, CHU de Caen Basse Normandie, Avenue Côte de Nacre, 14033, Caen Cedex, France

    Elsa Maitre & Xavier Troussard

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  1. Jérôme Paillassa
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Xavier Troussard. Consultant for Innate Pharma, Astra Zeneca, Abbvie, Beigne. Jérôme Paillassa et Elsa Maitre. The authors declare no conflict of interest.

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Paillassa, J., Maitre, E. & Troussard, X. Hairy Cell Leukemia (HCL) and HCL Variant: Updates and Spotlights on Therapeutic Advances. Curr Oncol Rep 24, 1133–1143 (2022). https://doi.org/10.1007/s11912-022-01285-1

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