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欧洲癌症研究与治疗组织(EORTC)原发性皮肤淋巴瘤分类:与修订的欧美淋巴瘤(R.E.A.L.)分类及提议的世界卫生组织(WHO)分类的比较

EORTC classification for primary cutaneous lymphomas: a comparison with the R.E.A.L. Classification and the proposed WHO Classification.

作者信息

Willemze R, Meijer C J

机构信息

Department of Dermatology, Free University Hospital, Amsterdam, The Netherlands.

出版信息

Ann Oncol. 2000;11 Suppl 1:11-5.

Abstract

Primary cutaneous lymphomas differ significantly from their nodal equivalents in clinical behaviour and prognosis, and often require a different therapeutic approach. Since currently used classification systems for non-Hodgkin lymphomas do not or insufficiently recognize the special character of these lymphomas, primary cutaneous lymphomas are not uncommonly diagnosed incorrectly, and/or treated inappropriately with unnecessarily aggressive therapies. For that reason the Cutaneous Lymphoma Group of the European Organization for Research and Treatment of Cancer (EORTC) has recently proposed a separate classification for the group of primary cutaneous lymphomas. The EORTC Classification is consistently based on a combination of clinical, histological, immunophenotypical and genetic criteria, and includes well-defined and recognizable disease entities. It contains a limited number of cutaneous T-cell lymphomas and cutaneous B-cell lymphomas, which comprise more than 95% of all primary cutaneous lymphomas. The clinical significance of this classification has been validated by long-term follow-up data of more than 800 patients with a primary cutaneous lymphoma. The basic principles of the EORTC Classification will be presented, and current controversies between the EORTC Classification on the one hand, and the R.E.A.L. Classification and the proposed WHO Classification on the other will be discussed.

摘要

原发性皮肤淋巴瘤在临床行为和预后方面与其淋巴结型淋巴瘤有显著差异,通常需要不同的治疗方法。由于目前用于非霍奇金淋巴瘤的分类系统没有或没有充分认识到这些淋巴瘤的特殊性质,原发性皮肤淋巴瘤常常被错误诊断,和/或接受不必要的激进治疗而未得到恰当治疗。因此,欧洲癌症研究与治疗组织(EORTC)皮肤淋巴瘤研究组最近提出了一套针对原发性皮肤淋巴瘤的单独分类。EORTC分类始终基于临床、组织学、免疫表型和遗传学标准的综合考量,涵盖了明确且可识别的疾病实体。它包含数量有限的皮肤T细胞淋巴瘤和皮肤B细胞淋巴瘤,这两类淋巴瘤占所有原发性皮肤淋巴瘤的95%以上。超过800例原发性皮肤淋巴瘤患者的长期随访数据证实了该分类的临床意义。本文将介绍EORTC分类的基本原则,并讨论EORTC分类与REAL分类以及WHO拟议分类之间目前存在的争议。

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