Detangling the Threads of Hairy Cell Leukemia, Beyond the Morphology and Into the Molecular.

Autor: Brazel D; Division of Hematology and Oncology, Scripps Clinic, La Jolla CA. Electronic address: brazel.danielle@scrippshealth.org., Hermel D; Division of Hematology and Oncology, Scripps Clinic, La Jolla CA., Gandhi P; Division of Pathology, Scripps Green Hospital, La Jolla CA., Saven A; Division of Hematology and Oncology, Scripps Clinic, La Jolla CA.
Jazyk: angličtina
Zdroj: Clinical lymphoma, myeloma & leukemia [Clin Lymphoma Myeloma Leuk] 2024 Sep; Vol. 24 (9), pp. 583-591. Date of Electronic Publication: 2024 May 03.
DOI: 10.1016/j.clml.2024.04.008
Abstrakt: Hairy cell leukemia (HCL) makes up 2% of leukemias in the United States and encompasses great molecular heterogeneity. The standard treatment paradigm involves purine nucleoside analogues in the upfront setting with high complete response rate to initial therapy but frequent relapses. There is an increasing role for BRAF inhibitors, with or without rituximab, in refractory and even in untreated patients. The response to purine analogues in HCL variant cases, otherwise classified as splenic lymphoma with prominent nucleolus in the 5th WHO edition classification, is less robust. Several antibodies, small molecular inhibitors, and combination regimens have been explored in HCL but data is frequently limited by case reports or small case series. Here we review available treatment options including their efficacy and safety profiles. We also explore investigational agents and potential future targets. The goal is to present a comprehensive therapeutic review of this rare disease entity and outline the ever increasing and novel therapeutic management options which interrupt key pathways in the pathogenesis of this malignancy.
Competing Interests: Disclosure The authors declare they have no competing interests.
(Copyright © 2024 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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