Most Hairy Cell Leukemia patients have a BRAF-V600E mutation.
Updated consensus guidelines for the diagnosis and management of patients with HCL and HCL variant.
Clive S Zent et al. · Blood · 2026
Background
Hairy cell leukemia (HCL) and HCL variant (HCLv) are distinct, rare, and chronic splenic B-cell lymphomas/leukemias that partially overlap in clinicopathologic presentation but differ in genetic basis, prognosis, and management.
Results
most Hairy Cell Leukemia patients have a specific gene change causing their cancer
More results
In contrast, HCLv lacks BRAFV600E mutation, requires combined therapy with purine analogues in addition to rituximab, and generally shows less durable responses.
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Conclusion
Here, an international team of hematologists, experts on these rare diseases, was convened by the Hairy Cell Leukemia Foundation to update the previous guidelines (published in 2017) by providing a summary of current methods to diagnose and manage patients with HCL and HCLv as well as a prospective on newer targeted therapies to further improve outcomes.