%0 Journal Article
%T Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study.
%A Bozonnat A
%A Beylot-Barry M
%A Dereure O
%A D'Incan M
%A Quereux G
%A Guenova E
%A Perier-Muzet M
%A Dalle S
%A Grange F
%A Viguier MA
%A Ram-Wolff C
%A Feldmeyer L
%A Beltraminelli H
%A Bonnet N
%A Amatore F
%A Maubec E
%A Franck N
%A Machet L
%A Chasset F
%A Brunet-Possenti F
%A Bouaziz JD
%A Battistella M
%A Donzel M
%A Pham-Ledard A
%A Bejar C
%A Moins-Teisserenc H
%A Mourah S
%A Saiag P
%A Hainaut E
%A Michel C
%A Bens G
%A Adamski H
%A Aubin F
%A Boulinguez S
%A Joly P
%A Tedbirt B
%A Templier I
%A Troin L
%A Montaudié H
%A Ingen-Housz-Oro S
%A Faiz S
%A Mortier L
%A Dobos G
%A Bagot M
%A Resche-Rigon M
%A Montlahuc C
%A Serret-Larmande A
%A de Masson A
%A  
%J EClinicalMedicine
%V 73
%N 0
%D 2024 Jul
%M 39007062
%F 17.033
%R 10.1016/j.eclinm.2024.102679
%X <B>UNASSIGNED: </B>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.<BR><B>UNASSIGNED: </B>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).<BR><B>UNASSIGNED: </B>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).<BR><B>UNASSIGNED: </B>Treatment with mogamulizumab was significantly and independently associated with decreased mortality in Sézary syndrome.<BR><B>UNASSIGNED: </B>French Society of Dermatology, Swiss National Science Foundation (IZLIZ3_200253/1) and SKINTEGRITY.CH collaborative research program.