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Long-term clinical outcome of a weekly 2-chlorodeoxyadenosine regimen in treatment-naïve patients with hairy cell leukemia.

Ann Hematol

January 2025

Department of Hematology, Seoul St. Mary's Hematology Hospital, College of Medicine, The Catholic University of Korea, Banpo-daero 222, Seocho-Gu, Seoul, South Korea.

Hairy cell leukemia (HCL) has a favorable clinical outcome with appropriate treatment; however, further research is needed on managing patients with relapsed or refractory disease and the risk of infection during prolonged periods. This study examined the long-term effects of 2-chlorodeoxyadenosine (2-CdA), particularly using a weekly infusion protocol, in treatment-naïve patients with HCL. This retrospective study evaluated the long-term follow-up data from 21 South Korean patients diagnosed with HCL.

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We found 18 patients with immunophenotype consistent with classic hairy cell leukemia (HCL) and BRAF mutations other than just V600E. Twelve had 1 non-V600E BRAF mutation and 6 had V600E with 1 (n=5) or 2 (n=1) non-V600E BRAF co-mutations.

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Hairy cell proliferations represent very different entities. They include hairy cell leukemia in its classic form (HCL), a well-defined entity, but also the variant form of HCL (LT-V ou HCL-V), whose presentation is far from HCL and whose prognosis is poorer. Other hairy cell proliferations include splenic red pulp lymphoma (SDRPL) and splenic marginal zone lymphomas (SMZL) with circulating villous cells.

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Pure Biclonal Hairy Cell Leukemia-Apt Diagnosis with Multicolor Flow Cytometry.

Int J Hematol Oncol Stem Cell Res

October 2024

Department of Pathology and Laboratory Medicine, Aga Khan University, Karachi, Pakistan.

Hairy cell leukemia (HCL) is a rare B-cell neoplasm that constitutes around 2 percent of all lymphoid leukemias and occurs more frequently in elderly males. The usual triad of HCL includes pancytopenia, splenomegaly, and hairy cells in the bone marrow. This is a case of an atypical presentation of biclonal HCL diagnosed on flow cytometry; the existence of biclonal HCL is extremely rare with very few case reports.

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