EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2023.

On behalf of the EORTC Cutaneous Lymphoma Tumours Group (EORTC-CLTG) and following up on earlier versions published in 2006 and 2017 this document provides an updated standard for the treatment of mycosis fungoides and Sézary syndrome (MF/SS). It considers recent relevant publications and treatment options introduced into clinical practice after 2017. Consensus was established among the authors through a series of consecutive consultations in writing and a round of discussion. Treatment options are assigned to each disease stage and, whenever possible and clinically useful, separated into first- and second line options annotated with levels of evidence. Major changes to the previous version include the incorporation of chlormethine, brentuximab vedotin, and mogamulizumab, recommendations on the use of pegylated interferon α (after withdrawal of recombinant unpegylated interferons), and the addition of paragraphs on supportive therapy and on the care of older patients. Still, skin-directed therapies are the most appropriate option for early-stage MF and most patients have a normal life expectancy but may suffer morbidity and impaired quality of life. In advanced disease treatment options have expanded recently. Most patients receive multiple consecutive therapies with treatments often having a relatively short duration of response. For those patients prognosis is still poor and only for a highly selected subset long term remission can be achieved with allogeneic stem cell transplantation. Understanding of the disease, its epidemiology and clinical course, and its most appropriate management are gradually advancing, and there is well-founded hope that this will lead to further improvements in the care of patients with MF/SS.

Informations de copyright

Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Johanna Latzka: speakers fees and travel support from Kyowa Kirin. Chalid Assaf: Advisory board: 4SC, Takeda, Helsinn, Innate Pharma, Recordati Rare Diseases, Kyowa Kirin. Martine Bagot: Advisory boards: Helsinn, Recordati, Innate Pharma, Kyowa Kirin, Takeda. Antonio Cozzio: Advisory activity for Kyowa Kirin, Takeda. Reinhard Dummer: has intermittent, project focused consulting and/or advisory relationships with Novartis, Merck Sharp & Dhome (MSD), Bristol-Myers Squibb (BMS), Roche, Amgen, Takeda, Pierre Fabre, Sun Pharma, Sanofi, Catalym, Second Genome, Regeneron, Alligator, T3 Pharma, MaxiVAX SA, Pfizer, Simcere and touchIME outside the submitted work. Emmanuella Guenova: member of the scientific advisory board of Scailyte AG; received grants and personal fees from Helsinn Healthcare SA and Takeda Pharmaceutical Company, personal fees from Mallinckrodt Pharmaceuticals, Kyowa Hakko Kirin Co., Ltd., Novartis, and Sanofi. Robert Gniadecki: reports carrying out clinical trials for Bausch Health, AbbVie and Janssen and has received honoraria as consultant and/or speaker from AbbVie, Bausch Health, Eli Lilly, Janssen, Mallincrodt, Novartis, Kyowa Kirin, Sun Pharma and Sanofi. Emmilia Hodak: received consulting and lecture fees from Takeda, consulting and lecture fees and travel support from Helsinn, lecture fees and travel support from Rafa. Constanze Jonak: received personal fees from Kyowa Kirin, Recordati Rare Diseases, and Takeda; Investigator for Innate Pharma and 4SC (grant paid to institution). Claus-Detlev Klemke: received travel support, lecture and consultancy fees from Actelion, Innate Pharma, Kyowa Kirin, Recordati Rare Diseases, Stemline and Takeda. Robert Knobler: Speaker for Mallinckrodt. Stephen Morris: none. Jan Peter Nicolay: received consulting and lecture fees from Actelion, Innate Pharma, Kyowa Kirin, Recordati, Takeda, TEVA; received a research grant from Kyowa Kirin paid to institution. Pablo Luis Ortiz Romero: received fees for Advisory committees from 4SC, Takeda, Kyowa Kirin, Actelion, Recordati rare diseases, Miragen, Bioprofarma, Mallinckrodt. Innate Pharma. Evangelia Papadavid: Advisory, speaker and grants: Recordati, Mallinckrodt, Takeda, UCB. Nicola Pimpinelli: received consulting fee from Actelion, lecture and advisory board fees from Kyowa Kirin, Recordati Rare Diseases, Takeda. Pietro Quaglino: participation to advisory board; received speaker fees from Therakos, Kyowa Kirin, Takeda, Cellgene, Helsinn-Recordati, 4SC. Annamari Ranki: none. Julia Scariscbrick: Takeda, Mallinckrodt, Kyowa Kirin, Recordati, Helsinn. Rudolf Stadler: received consult fees from Kyowa Kirin, Therakos, 4SC, Actelion. Liisa Väkevä: none. Marten Vermeer: unrestricted grants from: Kyowa, Takeda, Recordati, safety board: Innate; advisory board: Kyowa Kirin, Galderma, Speakers fee: Takeda, Kyowa. Ulrike Wehkamp: Consultancies for Takeda, Kyowa Kirin, Helsinn Healthcare, Mundipharma, Stemline and Galderma, and lectured at educational events sponsored by MSD, Takeda, Galderma, Kyowa Kirin, Stemline and Helsinn Healthcare all outside of the submitted work. Sean Whittaker: advisory boards for Kyowa Kirin and Recordati. Rein Willemze: none. Franz Trautinger: received consulting and lecture fees from Kyowa Kirin, Recordati Rare Diseases and Takeda.