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三十年皮肤淋巴瘤研究进展。

Thirty years of progress in cutaneous lymphoma research.

机构信息

Department of Dermatology, Leiden University Medical Center, Leiden, the Netherlands.

出版信息

G Ital Dermatol Venereol. 2012 Dec;147(6):515-21.

Abstract

Primary cutaneous lymphomas are a heterogeneous group of T-cell and B-cell neoplasms that present in the skin without any evidence of extracutaneous disease at the time of diagnosis. In 1980 primary cutaneous lymphomas other than mycosis fungoides, Sezary syndrome and some related disorders, collectively termed cutaneous T-cell lymphoma had not yet been recognized. This historic review describes the history of cutaneous lymphoma research in Europe over the last thirty years. European collaborative studies by dermatologists and pathologists, often coordinated by the European Organization for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Group, resulted in the definition of new types of cutaneous T-cell lymphomas (CTCL) and cutaneous B-cell lymphomas (CBCL), and the development of consensus classifications for primary cutaneous lymphomas (EORTC classification; WHO-EORTC classification; WHO classification 2008), which resulted in better diagnosis and treatment of these diseases. More recent activities described herein are the development of guidelines for staging, treatment and the design of clinical trials, often in close collaboration with the International Society for Cutaneous Lymphomas, the formation of an EORTC CTCL trials platform and attempts to translate the results of molecular genetic studies into clinical practise. In this review the importance of a multidisciplinary approach and continued international collaboration not only in clinical trials and guideline development, but also in basic research is emphasized.

摘要

原发性皮肤淋巴瘤是一组异质性的 T 细胞和 B 细胞肿瘤,在诊断时没有任何皮肤外疾病的证据。1980 年,除蕈样真菌病、Sezary 综合征和一些相关疾病外,其他原发性皮肤淋巴瘤尚未被认识。这篇历史性的综述描述了过去三十年欧洲皮肤淋巴瘤研究的历史。皮肤科医生和病理学家的欧洲合作研究,通常由欧洲癌症研究与治疗组织(EORTC)皮肤淋巴瘤组协调,导致了新的皮肤 T 细胞淋巴瘤(CTCL)和皮肤 B 细胞淋巴瘤(CBCL)类型的定义,并制定了原发性皮肤淋巴瘤的共识分类(EORTC 分类;WHO-EORTC 分类;WHO 2008 分类),这导致了这些疾病的更好的诊断和治疗。本文中描述的更近期的活动是制定分期、治疗和临床试验设计的指南,通常与国际皮肤淋巴瘤学会密切合作,形成 EORTC CTCL 试验平台,并尝试将分子遗传学研究的结果转化为临床实践。在这篇综述中,强调了多学科方法和持续的国际合作的重要性,不仅在临床试验和指南制定中,而且在基础研究中也是如此。

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