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Abstract：
In order to provide a common standard for the treatment of mycosis fungoides (MF) and Sézary syndrome (SS), the European Organisation for Research and Treatment of Cancer-Cutaneous Lymphoma Task Force (EORTC-CLTF) published in 2006 its consensus recommendations for the stage-adapted selection of management options for these neoplasms. Since then, the understanding of the pathophysiology and epidemiology of MF/SS has advanced, the staging system has been revised, new outcome data have been published and novel treatment options have been introduced. The purpose of the present document is to update the original recommendations bearing in mind that there are still only a limited number of controlled studies to support treatment decisions for MF/SS and that often treatment is determined by institutional experience and availability. This consensus on treatment recommendations was established among the authors through a series of consecutive consultations in writing and a round of discussion. Recommended treatment options are presented according to disease stage, whenever possible categorised into first- and second-line options and supported with levels of evidence as devised by the Oxford Centre for Evidence-Based Medicine (OCEBM). Skin-directed therapies are still the most appropriate option for early-stage MF, and most patients can look forward to a normal life expectancy. For patients with advanced disease, prognosis is still grim, and only for a highly selected subset of patients, prolonged survival can be achieved with allogeneic stem cell transplantation (alloSCT). There is a high need for the development and investigation in controlled clinical trials of treatment options that are based on our increasing understanding of the molecular pathology of MF/SS.
摘要：
为了提供治疗真菌病（MF）和Sézary综合征（SS）的共同标准，欧洲癌症-皮肤淋巴瘤研究和治疗组织特别工作组(EORTC-CLTF)于2006年发表了其关于这些肿瘤的治疗方案的阶段性适应选择的共识建议.从那以后,对MF/SS的病理生理学和流行病学的认识有了进步，暂存系统已经修改，新的结局数据已经公布,新的治疗方案也被引入.本文件的目的是更新最初的建议，同时铭记仍然只有数量有限的对照研究来支持MF/SS的治疗决定，并且治疗通常取决于机构的经验和可用性。通过一系列连续的书面咨询和一轮讨论，在作者之间建立了关于治疗建议的共识。根据疾病阶段提出推荐的治疗方案，在可能的情况下，将其分为一线和二线选项，并得到牛津循证医学中心（OCEBM）设计的证据水平的支持。针对皮肤的治疗仍然是早期MF的最合适选择，大多数患者可以期待正常的预期寿命。对于晚期疾病的患者，预后依然严峻,并且仅针对高度选择的患者子集，异基因干细胞移植（alloSCT）可以延长存活时间。基于我们对MF/SS的分子病理学的日益理解,对治疗选择的对照临床试验的开发和研究存在高度需求。
