Abstract

This article provides a brief update on the recommended diagnosis and treatment strategies for patients with the classic form of hairy cell leukemia (HCL) and HCL variant (HCLv). HCL is a chronic B-cell malignancy with multiple treatment options. In recent years, many novel drugs have been assessed for HCL treatment with promising results. The investigated nonchemotherapy options include moxetumomab pasudotox, which targets CD22; vemurafenib or dabrafenib, which target the BRAFV600E protein; trametinib, which targets mitogen-activated protein kinase enzyme; and ibrutinib, which targets Bruton tyrosine kinase. Purine analogs significantly improve survival in patients with HCL. However, patients often relapse, require multiple treatments, and may become refractory. The introduction of novel agents has expanded the spectrum of therapy possibilities in those patients. In the coming years, they will assist standard therapy for patients with HCL who may currently have suboptimal results.

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