EClinicalMedicine (Jul 2024)

Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort studyResearch in context

  • Alizée Bozonnat,
  • Marie Beylot-Barry,
  • Olivier Dereure,
  • Michel D’Incan,
  • Gaëlle Quereux,
  • Emmanuella Guenova,
  • Marie Perier-Muzet,
  • Stephane Dalle,
  • Florent Grange,
  • Manuelle-Anne Viguier,
  • Caroline Ram-Wolff,
  • Laurence Feldmeyer,
  • Helmut Beltraminelli,
  • Nathalie Bonnet,
  • Florent Amatore,
  • Eve Maubec,
  • Nathalie Franck,
  • Laurent Machet,
  • François Chasset,
  • Florence Brunet-Possenti,
  • Jean-David Bouaziz,
  • Maxime Battistella,
  • Marie Donzel,
  • Anne Pham-Ledard,
  • Claudia Bejar,
  • Hélène Moins-Teisserenc,
  • Samia Mourah,
  • Philippe Saiag,
  • Ewa Hainaut,
  • Catherine Michel,
  • Guido Bens,
  • Henri Adamski,
  • François Aubin,
  • Serge Boulinguez,
  • Pascal Joly,
  • Billal Tedbirt,
  • Isabelle Templier,
  • Laura Troin,
  • Henri Montaudié,
  • Saskia Ingen-Housz-Oro,
  • Sarah Faiz,
  • Laurent Mortier,
  • Gabor Dobos,
  • Martine Bagot,
  • Matthieu Resche-Rigon,
  • Claire Montlahuc,
  • Arnaud Serret-Larmande,
  • Adèle de Masson

Journal volume & issue
Vol. 73
p. 102679

Abstract

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Summary: 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.

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