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Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up

Jérôme Paillassa 1 Edouard Cornet 2 Stéphanie Noël 3 Cécile Tomowiak 3 Stéphane Lepretre 4 Sandrine Vaudaux 4 Jehan Dupuis 5 Alain Devidas 6 Bertrand Joly 6 Charlotte Petitdidier-Lionnet 6 Stéphanie Haiat 6 Clara Mariette 7 Catherine Thieblemont 8 Didier Decaudin 9 Patricia Validire-Charpy 9 Bernard Drenou 10 Jean-Claude Eisenmann 10 Mario Ojeda Uribe 10 Agnès Olivrie 11 Mohamed Touati 11 Olivier Lambotte 12 Olivier Hermine 13 Jean-Michel Karsenti 14 Pierre Feugier 15 Willy Vaillant 16 Jean Gutnecht 17 Eric Lippert 18 Fabienne Huysman 19 Kamel Ghomari 19 Marouane Boubaya 20 Vincent Levy 20 Jérémie Riou 21 Gandhi Damaj 2 Aline Schmidt 22, 1, 23 Mathilde Hunault-Berger 22, 1, 23 Xavier Troussard 2, * 
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.
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Jérôme Paillassa, Edouard Cornet, Stéphanie Noël, Cécile Tomowiak, Stéphane Lepretre, et al.. Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up. Blood Cancer Journal, Nature Publishing Group, 2020, 10 (5), pp.62. ⟨10.1038/s41408-020-0328-z⟩. ⟨inserm-03495791⟩

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