原发性皮肤淋巴瘤WHO-EORTC分类最新进展解读

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

原发性皮肤淋巴瘤(primarycutaneouslymphoma,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.

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(WHOEORTC)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.