Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Jun 21:73:102679.
doi: 10.1016/j.eclinm.2024.102679. eCollection 2024 Jul.

Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study

Alizée Bozonnat  1   2   3 , Marie Beylot-Barry  4 , Olivier Dereure  5 , Michel D'Incan  6 , Gaëlle Quereux  7 , Emmanuella Guenova  8 , Marie Perier-Muzet  9 , Stephane Dalle  9 , Florent Grange  10 , Manuelle-Anne Viguier  11 , Caroline Ram-Wolff  1 , Laurence Feldmeyer  12 , Helmut Beltraminelli  12 , Nathalie Bonnet  13 , Florent Amatore  13 , Eve Maubec  14 , Nathalie Franck  15 , Laurent Machet  16 , François Chasset  17 , Florence Brunet-Possenti  18 , Jean-David Bouaziz  1   2   3 , Maxime Battistella  2   3   19 , Marie Donzel  20 , Anne Pham-Ledard  4 , Claudia Bejar  14 , Hélène Moins-Teisserenc  3   21 , Samia Mourah  2   3   22 , Philippe Saiag  23 , Ewa Hainaut  24 , Catherine Michel  25 , Guido Bens  26 , Henri Adamski  27 , François Aubin  28 , Serge Boulinguez  29 , Pascal Joly  30 , Billal Tedbirt  30 , Isabelle Templier  31 , Laura Troin  32 , Henri Montaudié  32 , Saskia Ingen-Housz-Oro  33 , Sarah Faiz  34 , Laurent Mortier  34 , Gabor Dobos  35 , Martine Bagot  1   2   3 , Matthieu Resche-Rigon  2   3   36 , Claire Montlahuc  36 , Arnaud Serret-Larmande  2   3   36 , Adèle de Masson  1   2   3 ; Cutaneous Lymphomas French Study Group
Affiliations

Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study

Alizée Bozonnat et al. EClinicalMedicine. .

Erratum in

  • Erratum: Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study.
    Bozonnat A, Beylot-Barry M, Dereure O, D'Incan M, Quereux G, Guenova E, Perier-Muzet M, Dalle S, Grange F, Viguier MA, Ram-Wolff C, Feldmeyer L, Beltraminelli H, Bonnet N, Amatore F, Maubec E, Franck N, Machet L, Chasset F, Brunet-Possenti F, Bouaziz JD, Battistella M, Donzel M, Pham-Ledard A, Bejar C, Moins-Teisserenc H, Mourah S, Saiag P, Hainaut E, Michel C, Bens G, Adamski H, Aubin F, Boulinguez S, Joly P, Tedbirt B, Templier I, Troin L, Montaudié H, Ingen-Housz-Oro S, Faiz S, Mortier L, Dobos G, Bagot M, Resche-Rigon M, Montlahuc C, Serret-Larmande A, de Masson A; Cutaneous Lymphomas French Study Group. Bozonnat A, et al. EClinicalMedicine. 2024 Dec 6;79:102979. doi: 10.1016/j.eclinm.2024.102979. eCollection 2025 Jan. EClinicalMedicine. 2024. PMID: 39720605 Free PMC article.

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.

Keywords: Cutaneous T-cell lymphoma; Immunotherapy; Mogamulizumab; Monoclonal antibody; Sézary syndrome.

PubMed Disclaimer

Conflict of interest statement

AdM declares nonfinancial support from Kyowa Kirin and Recordati Rare Diseases; fees from Takeda, Almirall and Recordati Rare Diseases, and research funding, outside the scope of this study, from Kyowa Kirin, Innate Pharma, Almirall and Takeda. MB declares consultant fees from Innate Pharma, Kyowa Kirin, Takeda, BMS, Sanofi, Quantum Genomics, and research funding from Kyowa Kirin and Takeda, outside the scope of this study. SM declares consultant fees outside the scope of this study, from Pierre Fabre, Sanofi, Novartis and Biocartis, and has received research funding from BMS, Novartis and Roche. NF declares having received nonfinancial support from Kyowa Kirin. PS received a research grant from Pierre Fabre, fees unrelated to this manuscript from Bristol-Myers Squibb, MSD, Merck-Serono, Pfizer, Roche-Genentech, Pierre Fabre, and Novartis; received nonfinancial support from Bristol-Myers Squibb, MSD, Roche-Genentech, Pierre Fabre, and Novartis outside of the scope of this study. MBB declares consultant fees from Kyowa Kirin, Takeda and Recordati and research funding from Kyowa Kirin and Almirall outside the scope of this study. HMT declares consultant fees outside from the scope of this study from Innate Pharma, and has received research funding, outside the scope of this study, from Kyowa Kirin. SIHO consultant fees outside the scope of this study from Takeda and Recordati. GD declares consultant fees outside of the scope of this study from Kyowa Kirin and Recordati. EG declares consultant fees and/or grant support from Mallinckrodt, Helsinn, Takeda, Novartis and Kyowa Kirin unrelated to this work. FG declares consultant fees from Recordati and Kyowa Kirin, outside from the scope of this study. GQ declares consultant fees from Takeda, Recordati and Kyowa Kirin, outside the scope of this study. CM declares nonfinancial support from MSD, Pfizer, Novartis, Bristol-Myers Squibb, Pierre Fabre, Leo Pharma, Sanofi Aventis, Jannsen Cilag outside of the scope of this study. SB declares having received nonfinancial support from Kyowa Kirin and Recordati. MBag declares consulting fees from Kyowa Kirin, Takeda, Recordati. HB declares consultant fees from Kyowa Kirin. EH declares consultant fees outside the scope of this study from Bristol-Myers Squibb, Takeda, Sanofi, Jannsen Cilag, Blueprint Medicines, AbbVie and nonfinancial support from Kyowa Kirin, MSD, UCB Pharma, Novartis, Almirall, Pierre Fabre. The other authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1
Overall survival in the whole cohort of 339 patients, according to the time period and to the use of monoclonal antibodies in the first five lines of treatments. A. Overall survival in 339 patients. Overall survival was estimated from the date of diagnosis by the Kaplan–Meier method (dashed lines, 95% confidence interval). B. Overall survival in 339 patients according to the period of diagnosis. No significant difference was observed in terms of overall survival according to the time period. C. Overall survival according to the use, or not, of a therapeutic monoclonal antibody in the first 5 lines of treatments, analyzed in the subgroup of patients who had received at least 5 successive lines of treatments. The median number of consecutive treatment lines received by the patients was 4 (range, 0 to 14).
Fig. 2
Fig. 2
Overall survival according to the treatments received by the patients. A. Overall survival according to the use, or not, of mogamulizumab at any time point during the course of the disease. B. Overall survival according to the use, or not, of extracorporeal photopheresis at any time point during the course of the disease. C. Overall survival according to the use, or not, of interferon alfa at any time point during the course of the disease. Treatments were analyzed as time-dependent variables and numbers at risk not indicated for this reason. Hazard ratios, 95% confidence intervals and p-values are from univariable Cox analysis.
See this image and copyright information in PMC

References

    1. Dobos G., Pohrt A., Ram-Wolff C., et al. Epidemiology of cutaneous T-cell lymphomas: a systematic review and meta-analysis of 16,953 patients. Cancers. 2020;12 - PMC - PubMed
    1. Dobos G., de Masson A., Ram-Wolff C., et al. Epidemiological changes in cutaneous lymphomas: an analysis of 8593 patients from the French Cutaneous Lymphoma Registry. Br J Dermatol. 2020;184:1059. doi: 10.1111/bjd.19644. published online Oct 31. - DOI - PubMed
    1. Willemze R., Cerroni L., Kempf W., et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood. 2019;133:1703–1714. - PMC - PubMed
    1. Blaizot R., Ouattara E., Fauconneau A., Beylot-Barry M., Pham-Ledard A. Infectious events and associated risk factors in mycosis fungoides/Sézary syndrome: a retrospective cohort study. Br J Dermatol. 2018;179:1322–1328. - PubMed
    1. Ottevanger R., van Beugen S., Evers A.W.M., Willemze R., Vermeer M.H., Quint K.D. Quality of life in patients with Mycosis Fungoides and Sézary Syndrome: a systematic review of the literature. J Eur Acad Dermatol Venereol. 2021;35:2377–2387. - PMC - PubMed

Associated data