Epidemiology, clinical picture and long‐term outcomes of FIP1L1‐PDGFRA ‐positive myeloid neoplasm with eosinophilia: Data from 151 patients - Université Claude Bernard Lyon 1 Accéder directement au contenu
Article Dans Une Revue American Journal of Hematology Année : 2020

Epidemiology, clinical picture and long‐term outcomes of FIP1L1‐PDGFRA ‐positive myeloid neoplasm with eosinophilia: Data from 151 patients

1 CEREO - Centre de Référence National des Syndromes Hyperéosinophiliques
2 Hôpital Foch [Suresnes]
3 Centre Hospitalier Annecy-Genevois [Saint-Julien-en-Genevois]
4 Laboratoire CERBA [Saint Ouen l'Aumône]
5 CHU Bordeaux
6 Saint Anne Military Teaching Hospital [Toulon, France]
7 AP-HP - Hopital Saint-Louis [AP-HP]
8 CH Annecy Genevois
9 National Coordinating Reference Center for Rare Pulmonary Diseases
10 IVPC - Infections Virales et Pathologie Comparée - UMR 754
11 IUCT Oncopole - UMR 1037 - Institut Universitaire du Cancer de Toulouse - Oncopole
12 CIML - Centre d'Immunologie de Marseille - Luminy
13 CHU Saint-Antoine [AP-HP]
14 CHU ST-E - Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne]
15 CHU Nantes - Centre Hospitalier Universitaire de Nantes
16 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
17 RESO - Centre National de Référence pour les Maladies Auto-immunes Rares [CHU Strasbourg]
18 CRCINA-ÉQUIPE 7 - Innate Immunity and Immunotherapy
19 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Ponchaillou]
20 INEM - UM 111 (UMR 8253 / U1151) - Institut Necker Enfants-Malades
21 IRFAC - Inserm U1113 - Interface de Recherche Fondamentale et Appliquée en Cancérologie
22 CHU Strasbourg - Centre Hospitalier Universitaire [Strasbourg]
23 CHU Sud Saint Pierre [Ile de la Réunion]
24 IPC - Institut Paoli-Calmettes
25 CHU Caen
26 UNICANCER/CRCL - Centre de Recherche en Cancérologie de Lyon
27 CHV - Centre Hospitalier de Versailles André Mignot
28 CeRAINO - Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Ouest de France
29 Hôpital Ambroise Paré [AP-HP]
30 Université Paris-Saclay
Audrey Bidet
  • Fonction : Auteur
Lionel Galicier
Ludovic Lhermitte
Dina Naguib
  • Fonction : Auteur

Résumé

FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia (F/P+ MN-eo) is a rare disease: robust epidemiological data are lacking and reported issues are scarce, of low sample-size and limited follow-up. Imatinib mesylate (IM) is highly efficient but no predictive factor of relapse after discontinuation has yet been identified. One hundred and fifty-one patients with F/P+ MN-eo (143 males; mean age at diagnosis 49 years; mean annual incidence: 0.18 case per million population) were included in this retrospective nationwide study involving all French laboratories who perform the search of F/P fusion gene (study period: 2003-2019). The main organs involved included the spleen (44%), skin (32%), lungs (30%), heart (19%) and central nervous system (9%). Serum vitamin B12 and tryptase levels were elevated in 74/79 (94%) and 45/57 (79%) patients, respectively, and none of the 31 patients initially treated with corticosteroids achieved complete hematologic remission. All 148 (98%) IM-treated patients achieved complete hematologic and molecular (when tested, n = 84) responses. Forty-six patients eventually discontinued IM, among whom 20 (57%) relapsed. In multivariate analysis, time to IM initiation (continuous HR: 1,01 [0.99-1,03]; P = .05) and duration of IM treatment (continuous HR: 0,97 [0,95-0,99]; P = .004) were independent factors of relapse after discontinuation of IM. After a mean follow-up of 80 (56) months, the 1, 5- and 10-year overall survival rates in IM-treated patients were 99%, 95% and 84% respectively. In F/P+ MN-eo, prompt initiation of IM and longer treatment durations may prevent relapses after discontinuation of IM.

Domaines

Cancer

Dates et versions

inserm-03496695 , version 1 (20-12-2021)

Identifiants

Citer

Julien Rohmer, Amélie Couteau‐chardon, Julie Trichereau, Kewin Panel, Cyrielle Gesquiere, et al.. Epidemiology, clinical picture and long‐term outcomes of FIP1L1‐PDGFRA ‐positive myeloid neoplasm with eosinophilia: Data from 151 patients. American Journal of Hematology, 2020, 95 (11), pp.1314-1323. ⟨10.1002/ajh.25945⟩. ⟨inserm-03496695⟩
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