Current Therapy and New Directions in the Treatment of Hairy Cell Leukemia: A Review.

Abstract

Hairy cell leukemia (HCL) is a chronic B-cell leukemia noted for an indolent course that ultimately results in cytopenias and massive splenomegaly. Whereas treatment with the nucleoside purine analogues cladribine and pentostatin results in lengthy remissions in nearly all patients with HCL, most patients will experience relapse while a small percentage of patients' disease fails to respond to therapy in the first place. Retreatment with a purine nucleoside analogue often leads to an effective but limited response. For decades, few other viable therapeutic options were available to these patients who required retreatment. Recently, new insights into the mechanism of disease of HCL have led to research in new potential treatment agents, either alone or with a purine nucleoside analogue. Clinical trials with rituximab, bendamustine, and conjugate immunotoxins will reveal what role these therapies will have in HCL treatment. A better understanding of the BRAF/MEK/ERK pathway and the B-cell signaling pathway has allowed further exploration into the novel drugs vemurafenib, dabrafenib, trametinib, and ibrutinib.

• Authors: Aditya Sarvaria, Zheng Topp, Alan Saven
• Journal: JAMA oncology (JAMA Oncol) Vol. 2 Issue 1 Pg. 123-9 (Jan 2016) ISSN: 2374-2445 [Electronic] United States
• PMID: 26513168 (Publication Type: Historical Article, Journal Article, Review)

Chemical References

• Antineoplastic Agents
• Biomarkers, Tumor

Topics

• Antineoplastic Agents (therapeutic use)
• Biomarkers, Tumor (genetics, metabolism)
• Diffusion of Innovation
• Drug Resistance
• History, 20th Century
• History, 21st Century
• Humans
• Leukemia, Hairy Cell (drug therapy, genetics, history, metabolism, mortality)
• Molecular Targeted Therapy (adverse effects, history, trends)
• Patient Selection
• Recurrence
• Signal Transduction (drug effects)
• Treatment Outcome