Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study
A. Bozonnat, M. Beylot‐Barry, O. Dereure, M. D’Incan, G. Quéreux, Emmanuella Guenova, Marie Perier‐Muzet, Stéphane Dalle, Florent Grange, M. Viguier, Caroline Ram-Wolff, Laurence Feldmeyer, Helmut Beltraminelli, Nathalie Bonnet, F. Amatore, E. Maubec, Nathalie Franck, L. Machet, François Chasset, F. Brunet‐Possenti, Jean-David Bouaziz, Maxime Battistella, Marie Donzel, Anne Pham-Ledard, C. Béjar, Hélène Moins-Teisserenc, Samia Mourah, Philippe Saïag, E. Hainaut, Catherine Michel, Guido Bens, Henri Adamski, F. Aubin, Serge Boulinguez, P. Joly, Billal Tedbirt, I. Templier, L. Troin, Henri Montaudié, S. Oro, Sarah Faiz, Laurent Mortier, Gábor Dobos, M. Bagot, Matthieu Resche‐Rigon, Claire Montlahuc, Arnaud Serret-Larmande, Adèle de Masson
Abstract
Background: 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.