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- Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up
Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up
Authors
Jerome Paillassa, Edouard Cornet, Stephanie Noel, Cecile Tomowiak, Stephane Lepretre, Sandrine Vaudaux, Jehan Dupuis, Alain Devidas, Bertrand Joly, Charlotte Petitdidier-Lionnet, Stephanie Haiat, Clara Mariette, Catherine Thieblemont, Didier Decaudin, Patricia Validire-Charpy, Bernard Drenou, Jean-Claude Eisenmann, Mario Ojeda Uribe, Agnès Olivrie, Mohamed Touati, Olivier Lambotte, Olivier Hermine, Jean-Michel Karsenti, Pierre Feugier, Willy Vaillant, Jean Gutnecht, Eric Lippert, Fabienne Huysman, Kamel Ghomari, Marouane Boubaya, Vincent Levy, Jeremie Riou, Gandhi Damaj, Aline Tanguy-Schmidt, Mathilde Hunault-Berger, Xavier Troussard
Abstract
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.