Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Mar 9:14:1797-1805.
doi: 10.2147/OTT.S242247. eCollection 2021.

Current and Emerging Therapeutic Options for Hairy Cell Leukemia Variant

Affiliations
Review

Current and Emerging Therapeutic Options for Hairy Cell Leukemia Variant

Qiuying Liu et al. Onco Targets Ther. .

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.

PubMed Disclaimer

Conflict of interest statement

QSL and NH report no conflicts of interest. NE is on Speaker’s Bureau for Verastem Oncology and Beigene; received honoraria from Pharmacyclics. LA provides consultation services for Innate Pharma. LAalso receives research funding from the Hairy Cell Leukemia Foundation. The authors report no other conflicts of interest in this work.

References

    1. Bouroncle BA, Wiseman BK, Doan CA. Leukemic reticuloendotheliosis. Blood. 1958;13(7):609–630. doi:10.1182/blood.V13.7.609.609 - DOI - PubMed
    1. Sarvaria A, Topp Z, Saven A. Current therapy and new directions in the treatment of hairy cell leukemia: a review. JAMA Oncol. 2016;2(1):123–129. doi:10.1001/jamaoncol.2015.4134 - DOI - PubMed
    1. Grever MR, Blachly JS, Andritsos LA. Hairy cell leukemia: update on molecular profiling and therapeutic advances. Blood Rev. 2014;28(5):197–203. doi:10.1016/j.blre.2014.06.003 - DOI - PubMed
    1. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375–2390. doi:10.1182/blood-2016-01-643569 - DOI - PMC - PubMed
    1. Kreitman RJ. Hairy cell leukemia: present and future directions. Leuk Lymphoma. 2019;60(12):2869–2879. doi:10.1080/10428194.2019.1608536 - DOI - PMC - PubMed

LinkOut - more resources