原发性皮肤淋巴瘤WHO-EORTC分类最新进展解读

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。

WHO-EORTC Classification of Primary Cutaneous Lymphomas:Updates and Interpretation

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.