Bozonnat, AlizéeAlizéeBozonnatBeylot-Barry, MarieMarieBeylot-BarryDereure, OlivierOlivierDereureD'Incan, MichelMichelD'IncanQuereux, GaëlleGaëlleQuereuxGuenova, EmmanuellaEmmanuellaGuenovaPerier-Muzet, MarieMariePerier-MuzetDalle, StephaneStephaneDalleGrange, FlorentFlorentGrangeViguier, Manuelle-AnneManuelle-AnneViguierRam-Wolff, CarolineCarolineRam-WolffFeldmeyer, LaurenceLaurenceFeldmeyerBeltraminelli, HelmutHelmutBeltraminelliBonnet, NathalieNathalieBonnetAmatore, FlorentFlorentAmatoreMaubec, EveEveMaubecFranck, NathalieNathalieFranckMachet, LaurentLaurentMachetChasset, FrançoisFrançoisChassetBrunet-Possenti, FlorenceFlorenceBrunet-PossentiBouaziz, Jean-DavidJean-DavidBouazizBattistella, MaximeMaximeBattistellaDonzel, MarieMarieDonzelPham-Ledard, AnneAnnePham-LedardBejar, ClaudiaClaudiaBejarMoins-Teisserenc, HélèneHélèneMoins-TeisserencMourah, SamiaSamiaMourahSaiag, PhilippePhilippeSaiagHainaut, EwaEwaHainautMichel, CatherineCatherineMichelBens, GuidoGuidoBensAdamski, HenriHenriAdamskiAubin, FrançoisFrançoisAubinBoulinguez, SergeSergeBoulinguezJoly, PascalPascalJolyTedbirt, BillalBillalTedbirtTemplier, IsabelleIsabelleTemplierTroin, LauraLauraTroinMontaudié, HenriHenriMontaudiéIngen-Housz-Oro, SaskiaSaskiaIngen-Housz-OroFaiz, SarahSarahFaizMortier, LaurentLaurentMortierDobos, GaborGaborDobosBagot, MartineMartineBagotResche-Rigon, MatthieuMatthieuResche-RigonMontlahuc, ClaireClaireMontlahucSerret-Larmande, ArnaudArnaudSerret-Larmandede Masson, AdèleAdèlede Masson2024-10-262024-10-262024-07https://boris-portal.unibe.ch/handle/20.500.12422/179161BACKGROUND Sézary syndrome is an extremely rare and fatal cutaneous T-cell lymphoma (CTCL). Mogamulizumab, an anti-CCR4 monoclonal antibody, has recently been associated with increased progression-free survival in a randomized clinical trial in CTCL. We aimed to evaluate OS and prognostic factors in Sézary syndrome, including treatment with mogamulizumab, in a real-life setting. METHODS Data from patients with Sézary (ISCL/EORTC stage IV) and pre-Sézary (stage IIIB) syndrome diagnosed from 2000 to 2020 were obtained from 24 centers in Europe. Age, disease stage, plasma lactate dehydrogenases levels, blood eosinophilia at diagnosis, large-cell transformation and treatment received were analyzed in a multivariable Cox proportional hazard ratio model. This study has been registered in ClinicalTrials (SURPASSe01 study: NCT05206045). FINDINGS Three hundred and thirty-nine patients were included (58% men, median age at diagnosis of 70 years, Q1-Q3, 61-79): 33 pre-Sézary (9.7% of 339), 296 Sézary syndrome (87.3%), of whom 10 (2.9%) had large-cell transformation. One hundred and ten patients received mogamulizumab. Median follow-up was 58 months (95% confidence interval [CI], 53-68). OS was 46.5% (95% CI, 40.6%-53.3%) at 5 years. Multivariable analysis showed that age ≥ 80 versus <50 (HR: 4.9, 95% CI, 2.1-11.2, p = 0.001), and large-cell transformation (HR: 2.8, 95% CI, 1.6-5.1, p = 0.001) were independent and significant factors associated with reduced OS. Mogamulizumab treatment was significantly associated with decreased mortality (HR: 0.34, 95% CI, 0.15-0.80, p = 0.013). INTERPRETATION Treatment with mogamulizumab was significantly and independently associated with decreased mortality in Sézary syndrome. FUNDING French Society of Dermatology, Swiss National Science Foundation (IZLIZ3_200253/1) and SKINTEGRITY.CH collaborative research program.enCutaneous T-cell lymphoma Immunotherapy Mogamulizumab Monoclonal antibody Sézary syndrome600 - Technology::610 - Medicine & healtharticle10.48350/1990153900706210.1016/j.eclinm.2024.102679