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

Jérôme Paillassa; Edouard Cornet; Stéphanie Noël; Cécile Tomowiak; Stéphane Lepretre; Sandrine Vaudaux; Jehan Dupuis; Alain Devidas; Bertrand Joly; Charlotte Petitdidier-Lionnet; Stéphanie 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; Jérémie Riou; Gandhi Damaj; Aline Schmidt; Mathilde Hunault-Berger; Xavier Troussard

doi:10.1038/s41408-020-0328-z

Journal articles

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, *}

* Corresponding author

1 CHU Angers - Centre Hospitalier Universitaire d'Angers

2 CHU Caen

3 CIC - Poitiers

4 GPMCND - Génomique et Médecine Personnalisée du Cancer et des Maladies Neuropsychiatriques

5 CHU Henri Mondor

6 CH Sud Francilien - Centre Hospitalier Sud Francilien [Corbeil-Essonnes]

7 CHU - Centre Hospitalier Universitaire [Grenoble]

8 AP-HP - Hopital Saint-Louis [AP-HP]

9 Institut Curie [Paris]

10 CH E.Muller Mulhouse - Centre Hospitalier Emile Muller [Mulhouse]

11 CHU Limoges

12 AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre)

13 CHU Necker - Enfants Malades [AP-HP]

14 CHU Nice - Centre Hospitalier Universitaire de Nice

15 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy

16 CH Auch - Centre Hospitalier d'Auch

17 CHI Fréjus - St Raphaël - Centre Hospitalier Intercommunal Fréjus - St Raphaël

18 CHU-Brest-Hemato - CHRU Brest - Service d'Hématologie

19 CH Beauvais

20 Hôpital Avicenne [AP-HP]

21 MINT - Micro et Nanomédecines Translationnelles

22 CRCINA-ÉQUIPE 7 - Innate Immunity and Immunotherapy

CRCINA - Centre de Recherche en Cancérologie et Immunologie Nantes-Angers

23 Fédération Hospitalo-Universitaire 'Grand Ouest Against Leukemia' (FHU GOAL)

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.

Document type :

Journal articles

Domain :

Life Sciences [q-bio] / Cancer

Complete list of metadata

https://www.hal.inserm.fr/inserm-03495791
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Submitted on : Monday, December 20, 2021 - 12:03:28 PM
Last modification on : Wednesday, January 19, 2022 - 5:08:05 PM

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

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

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