Hairy Cell Leukemia: a rare disease undergoing therapeutic transformation
Keywords:
Hairy cell leukemia, BRAF V600E mutation, immunotherapiesAbstract
Hairy cell leukemia is a rare hematologic neoplasm that has undergone significant advances in its diagnosis and treatment. This study aimed to characterize its main epidemiological, clinical, diagnostic, and therapeutic aspects through a literature review of 22 sources published in Spanish, English, and Portuguese over the past ten years. The reviewed evidence shows that this disease accounts for approximately 1% to 2% of lymphoid leukemias and is generally characterized by an indolent course. Its main clinical manifestations include cytopenias, splenomegaly, and increased susceptibility to infections. From a diagnostic perspective, the identification of the BRAF V600E mutation and the expression of molecular markers such as CD19, CD20, CD22, CD11, CD25, CD103, and CD123 are particularly relevant. Standard treatment is primarily based on purine analogs, which may be combined with rituximab according to the patient’s characteristics and clinical condition. Advances involving targeted immunotherapies for patients with refractory or relapsed disease were also identified. It is concluded that, although the low incidence of hairy cell leukemia limits the conduct of large-scale clinical trials, multicenter collaboration, long-term follow-up, and the development of targeted therapies may contribute to increasingly precise and potentially chemotherapy-free treatments.
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Copyright (c) 2026 Liliana Teruel-Leyva, Leonardo Silva-dos Santos, Daniela Stefania Gallardo-Ruiz, Doménica Cristina Ruiz-Mera, José Eduardo Orozco-Ruiz, Byron Allan Cachott-Brito
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