Department of Dermatology and Venereology, University Hospital of St. Pölten, Karl Landsteiner University of Health Sciences, St. Pölten, Austria; Karl Landsteiner Institute of Dermatological Research, Department of Dermatology and Venereology, University Hospital of St. Pölten, St. Pölten, Austria.
Department of Dermatology, HELIOS Klinikum Krefeld, Krefeld, Germany; Institute for Molecular Medicine, Medical School Hamburg, University of Applied Sciences and Medical University, Hamburg, Germany; Department of Dermatology, HELIOS Klinikum Schwerin, University Campus of The Medical School Hamburg, Schwerin, Germany.
Eur J Cancer. 2023 Dec;195:113343. doi: 10.1016/j.ejca.2023.113343. Epub 2023 Sep 18.
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.
代表 EORTC 皮肤淋巴瘤肿瘤学组（EORTC-CLTG），并在 2006 年和 2017 年发布的早期版本的基础上，本文提供了一个更新的蕈样肉芽肿和塞扎里综合征（MF/SS）治疗标准。它考虑了最近的相关出版物和 2017 年后引入临床实践的治疗选择。通过一系列连续的书面协商和一轮讨论，作者之间达成了共识。治疗选择分配给每个疾病阶段，并且在可能和临床有用的情况下，将其分为一线和二线选择，并附有证据水平的注释。与前一版本相比，主要的变化包括氯苯丁胺、 Brentuximab vedotin 和 mogamulizumab 的纳入，关于聚乙二醇化干扰素 α 的使用建议（在重组非聚乙二醇化干扰素撤出后），以及关于支持性治疗和老年患者护理的段落的添加。尽管如此，皮肤定向疗法仍然是早期 MF 最合适的选择，大多数患者的预期寿命正常，但可能会出现发病率和生活质量受损。在晚期疾病中，治疗选择最近有所扩大。大多数患者接受多次连续治疗，治疗的反应通常持续时间相对较短。对于这些患者，预后仍然很差，只有极少数患者可以通过异基因造血干细胞移植实现长期缓解。对疾病的理解、其流行病学和临床过程以及其最合适的管理正在逐步推进，并且有充分的理由希望这将进一步改善 MF/SS 患者的护理。
