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摘要: 原发性皮肤淋巴瘤(primary cutaneous lymphoma，PCL)是一类结外非霍奇金淋巴瘤，具有独特的临床表现和组织病理学特征。2005年世界卫生组织-欧洲癌症研究和治疗组织(World Health Organization-European Organization for Research and Treatment of Cancer，WHO-EORTC)发表的PCL分类是该病的重要诊断标准和分类依据。随着研究进展，2018年WHO-EORTC更新了PCL分类，新增了慢性活动性EB病毒感染、原发性皮肤肢端CD8+T细胞淋巴瘤、EB病毒阳性皮肤黏膜溃疡3种PCL类型，并对部分PCL的亚型进行了修订，其中涉及淋巴瘤样丘疹病、原发性皮肤边缘区淋巴瘤、原发性皮肤CD4+小/中T细胞淋巴组织增殖性疾病等亚型。本文对PCL WHO-EORTC分类的最新进展进行解读。
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关键词:
- 原发性皮肤淋巴瘤 /
- T细胞淋巴瘤 /
- B细胞淋巴瘤 /
- 结外NK/T细胞淋巴瘤
Abstract: Primary cutaneous lymphomas (PCL) are a group of non-Hodgkin's lymphomas that are presented with specific clinical manifestations and histopathological characteristics. The classification of PCL published by World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) in 2005 was the important diagnostic criteria and classification basis for cutaneous lymphomas. With research progression, WHO-EORTC updated the classification of PCL in 2018, adding three subtypes of PCL, namely, chronic active Epstein Barr virus (EBV) infection, primary cutaneous acral CD8+ T-cell lymphoma, and EBV-positive mucocutaneous ulcer. Besides, some subtypes of PCL were revised, including lymphomatoid papulosis, primary cutaneous marginal zone lymphoma, and primary cutaneous CD4+ small/medium T cell lymphoproliferative disease. This article interprets the update of WHO-EORTC classification of PCL.作者贡献： 刘洁负责文章选题和指南解读; 罗毅鑫和刘兆睿负责文章撰写; 刘跃华、张薇、卢朝辉负责参与指南解读及文章修订。利益冲突： 无 -
表 1 2005版和2018版WHO-EORTC原发性皮肤淋巴瘤分类比较
2005版 2018版 皮肤T细胞淋巴瘤 皮肤T细胞淋巴瘤 蕈样肉芽肿 蕈样肉芽肿 亲毛囊性蕈样肉芽肿 亲毛囊性蕈样肉芽肿 PAGET样网状细胞增生症 Paget样网状细胞增生症 肉芽肿性皮肤松弛症 肉芽肿性皮肤松弛症 Sézary综合症 Sézary综合症 成人T细胞白血病/淋巴瘤 成人T细胞白血病/淋巴瘤 原发性皮肤CD30+ T细胞淋巴增殖性疾病 原发性皮肤CD30+T细胞淋巴增殖性疾病 原发性皮肤间变大细胞淋巴瘤 原发性皮肤间变大细胞淋巴瘤 淋巴瘤样丘疹病 淋巴瘤样丘疹病* 皮下脂膜炎样T细胞淋巴瘤 皮下脂膜炎样T细胞淋巴瘤 结外NK/T细胞淋巴瘤，鼻型 结外NK/T细胞淋巴瘤，鼻型 原发性皮肤外周T细胞淋巴瘤，非特指型 慢性活动性EB病毒感染 原发性皮肤γ/δ T细胞淋巴瘤 原发性皮肤外周T细胞淋巴瘤，罕见亚型 原发性皮肤侵袭性亲表皮CD8+细胞淋巴瘤(暂定) 原发性皮肤γ/δ T细胞淋巴瘤 原发性皮肤CD4+小/中T细胞淋巴瘤(暂定) 原发性皮肤侵袭性亲表皮CD8+T细胞淋巴瘤(暂定) 皮肤B细胞淋巴瘤 原发性皮肤CD4+小/中T细胞淋巴组织增殖性疾病(暂定)* 原发性皮肤边缘区淋巴瘤 原发性皮肤肢端CD8+T细胞淋巴瘤(暂定) 原发性皮肤滤泡中心淋巴瘤 原发性皮肤外周T细胞淋巴瘤，非特指型 原发性皮肤弥漫性大B细胞淋巴瘤，腿型 皮肤B细胞淋巴瘤 原发性皮肤弥漫性大B细胞淋巴瘤，其他原发性皮肤边缘区淋巴瘤* 血管内大B细胞淋巴瘤 原发性皮肤滤泡中心淋巴瘤 血液系统肿瘤前体原发性皮肤弥漫性大B细胞淋巴瘤，腿型 CD4+/CD56+血液皮肤肿瘤(母细胞性NK细胞淋巴瘤)EB病毒阳性皮肤黏膜溃疡(暂定) 血管内大B细胞淋巴瘤 WHO-EORTC：世界卫生组织-欧洲癌症研究和治疗组织; A为删减内容; A为新增内容; *为修订内容
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