Remarkable Response of Hairy Cell Leukemia Variant to Single-Agent Cladribine.

ABSTRACT: Classical hairy cell leukemia (cHCL) and related mature lymphoid B-cell neoplasms including hairy cell leukemia variant (HCLv) and splenic diffuse red pulp lymphoma (SDRPL) are a rare subset of lymphoid neoplasms. cHCL accounts for around 2% of all leukemias and is characterized by a peripheral smear with large lymphoid cells with cytoplasmic projections giving the cells a hairy appearance, splenomegaly, and cytopenias. Majority of cHCL cases harbor a BRAFV600E mutation. cHCL usually responds well to single-agent purine analogs. HCLv is even rarer and constitutes around 0.4% of lymphoid malignancies. Unlike cHCL, HCLv is less responsive to standard single-agent purine analogs and typically does not harbor the BRAFV600E mutation. The "hairy cells," splenomegaly, and cytopenias are common in

SUBMITTER: Otieno SB 

PROVIDER: S-EPMC9188828 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature