Abstract：
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.
摘要：
本文件代表EORTC皮肤淋巴瘤肿瘤组（EORTC-CLTG），并对2006年和2017年发布的早期版本进行了跟进，为治疗真菌病和Sézary综合征（MF/SS）提供了更新的标准。它考虑了2017年后引入临床实践的最新相关出版物和治疗方案。通过一系列连续的书面磋商和一轮讨论，在作者之间建立了共识。治疗方案被分配到每个疾病阶段，只要有可能和临床上有用，分为第一行和第二行选项，注释有证据水平。对先前版本的主要更改包括掺入了氯甲因，Brentuximabvedotin,和莫加穆利珠单抗，关于使用聚乙二醇化干扰素α的建议（在重组非聚乙二醇化干扰素撤出后），并增加了关于支持治疗和老年患者护理的段落。尽管如此，针对皮肤的治疗是早期MF的最合适选择,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的问题.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,其中治疗通常具有相对短的响应持续时间。对于这些患者，预后仍然很差，只有高度选择的子集才能通过同种异体干细胞移植实现长期缓解。了解疾病，它的流行病学和临床课程，其最合适的管理正在逐步推进，并且有充分的希望，这将导致进一步改善MF/SS患者的护理。
