SUMMARY
Hairy cell leukaemia (HCL) is a rare hematologic malignancy with high response rates after purine-analogue based therapy and an excellent long-term prognosis. We reviewed 104 HCL patients diagnosed between 1980 and 2022 at the University Hospitals Leuven to analyse long-term outcomes and complications. Median follow-up was twelve years. In total, 96 patients (92%) received a first-line treatment consisting of splenectomy (n=13), interferon-α (n=13) and cladribine (n=70). This last therapy resulted in the best response rates (overall response (OR) 99%, complete response (CR) 71%) and a long-lasting progression-free-survival (median PFS ten years). Forty-three percent of patients received multiple therapies for subsequent relapses. The median number of treatment lines was one. The median overall survival (OS) was 30.8 years with a 5-year and 10-year OS of approximately 98% and 91%. Although the prognosis of HCL patients is very good, infections and second malignancies are frequently observed. In this cohort, 55% of the patients had a major infection with an infection-related mortality of 3%. After diagnosis, 22% of the HCL patients developed one or more second malignancies, ranging from 1–3 per patient, with a 10-year cumulative risk of 14.5%.
(BELG J HEMATOL 2024;15(6):249–56)
