Abstract
In total, 279 patients with hairy-cell leukemia (HCL) were analyzed, with a median follow-up of 10 years. Data were collected up to June 2018. We analyzed responses to treatment, relapses, survival, and the occurrence of second malignancies during follow-up. The median age was 59 years. In total, 208 patients (75%) were treated with purine analogs (PNAs), either cladribine (159) or pentosatin (49), as the first-line therapy. After a median follow-up of 127 months, the median overall survival was 27 years, and the median relapse-free survival (RFS) was 11 years. The cumulative 10-year relapse incidence was 39%. In patients receiving second-line therapy, the median RFS was 7 years. For the second-line therapy, using the same or another PNA was equivalent. We identified 68 second malignancies in 59 patients: 49 solid cancers and 19 hematological malignancies. The 10-year cumulative incidences of cancers, solid tumors, and hematological malignancies were 15%, 11%, and 5.0%, respectively, and the standardized incidence ratios were 2.22, 1.81, and 6.67, respectively. In multivariate analysis, PNA was not a risk factor for second malignancies. HCL patients have a good long-term prognosis. PNAs are the first-line treatment. HCL patients require long-term follow-up because of their relatively increased risk of second malignancies.
Highlights
Hairy cell leukemia (HCL) is a rare B-cell chronic lymphoproliferative disorder characterized by atypical lymphoid cells with hairy projections in the peripheral blood, bone marrow, spleen, and/or liver[1,2]
Twenty-one percent of patients presented had an infectious disease at the time of the diagnosis of HCL
In multivariate analysis (Supplementary Information 8), the percentage of hairy cells at diagnosis as a continuous variable (p = 0.006, HR 1.010, 95% Confidence intervals (CIs): 1.003; 1.018) and using other first-line treatments (p = 0.004, HR 2.533, 95% CI: 1.349; 4.754) were both predictors of a worse relapse-free survival (RFS), whereas achieving CR1 (p < 0.001, HR 0.355, 95% CI: 0.228; 0.555) was a predictor of a longer RFS
Summary
Hairy cell leukemia (HCL) is a rare B-cell chronic lymphoproliferative disorder characterized by atypical lymphoid cells with hairy projections in the peripheral blood, bone marrow, spleen, and/or liver[1,2]. The median RFS after first-line treatment was 11 years (136 months (95% CI: 109; 163)): 163, 159, 55, and 50 months after treatment with cladribine, pentostatin, other treatments and IFNα, respectively (Supplementary Information 7).
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