Current and Emerging Therapeutic Options for Hairy Cell Leukemia Variant

Onco Targets Ther. 2021 Mar 9:14:1797-1805. doi: 10.2147/OTT.S242247. eCollection 2021.

Abstract

Hairy cell leukemia variant (HCL-v) is a rare B-cell lymphoproliferative disorder with distinct immunophenotypic and molecular characteristics when compared to classical hairy cell leukemia (HCL-c). In contrast to the enormous progress in therapeutic options for HCL-c, HCL-v remains a therapeutic challenge due to inferior outcomes with standard chemoimmunotherapy and BCR signaling pathway inhibitors, and due to the fact that HCL-v has limited molecular therapeutic targets. In addition, because of the rarity of the disease, there is a paucity of later phase studies or multicenter trials to guide treatment decisions. In this article, we briefly review the diagnostic criteria and clinical characteristics of HCL-v and present a comprehensive overview of current therapeutic options in HCL-v.

Keywords: HCL-c; HCL-v; rare lymphoid malignancies; salvage therapy.

Publication types

  • Review