Fiche publication
Date publication
mars 2025
Journal
Leukemia
Auteurs
Membres identifiés du Cancéropôle Est :
Pr GIRODON François
Tous les auteurs :
Beauverd Y, Ianotto JC, Thaw KH, Sobas M, Sadjadian P, Curto-Garcia N, Shih LY, Devos T, Krochmalczyk D, Galli S, Bieniaszewska M, Seferynska I, McMullin MF, Armatys A, Spalek A, Waclaw J, Zdrenghea MT, Legros L, Girodon F, Lewandowski K, Bellosillo B, Samuelsson J, Abuin Blanco A, Cony-Makhoul P, Collins A, James C, Kusec R, Lauermannova M, Noya MS, Skowronek M, Szukalski L, Szmigielska-Kaplon A, Wondergem M, Dudchenko I, Gora-Tybor J, Laribi K, Kulikowska de Nałęcz A, Demory JL, Le Dû K, Zweegman S, Besses Raebel C, Skoda RC, Giraudier S, Griesshammer M, Kiladjian JJ, Harrison CN
Lien Pubmed
Résumé
Essential thrombocythemia (ET) and polycythemia vera (PV) are rare in adolescent and young adult (AYA). These conditions, similar to those in older patients, are linked with thrombotic complications and the potential progression to secondary myelofibrosis (sMF). This retrospective study of ET and PV patients diagnosed before age 25 evaluated complication rates and impact of cytoreductive drugs on outcomes. Among 348 patients (278 ET, 70 PV) with a median age of 20 years, the of thrombotic events was 1.9 per 100 patient-years. Risk factors for thrombosis included elevated white blood cell count (>11 × 10/L) (HR: 2.7, p = 0.012) and absence of splenomegaly at diagnosis (HR: 5.7, p = 0.026), while cytoreductive drugs did not reduce this risk. The incidence of sMF was 0.7 per 100 patient-years. CALR mutation (HR: 6.0, p < 0.001) and a history of thrombosis (HR: 3.8, p = 0.015) were associated with sMF risk. Interferon as a first-line treatment significantly improved myelofibrosis-free survival compared to other treatments or the absence of cytoreduction (p = 0.046). Although cytoreduction did not affect thrombotic event, early interferon use reduced sMF risk. These findings support interferon use to mitigate sMF risk in AYA ET and PV patients.
Référence
Leukemia. 2025 03 12;:
