Cutaneous EBV-related lymphoproliferative disorders.

Cutaneous EBV-related lymphoproliferative disorders.
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DOI:
10.1053/j.semdp.2016.11.003
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发表时间:
2017-01
影响因子:
2.3
通讯作者:
Jaffe ES
Jaffe ES
中科院分区:
医学3区
文献类型:
--
作者:
Gru AA;Jaffe ES

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本文将重点介绍与EBV相关的皮肤淋巴组织增生性疾病,重点介绍WHO造血系统肿瘤分类第4版修订版中即将发生的变化,其中许多涉及NK细胞或T细胞来源的皮肤疾病。结外NK/T细胞淋巴瘤通常发生在上呼吸消化道,但可继发于皮肤。儿童年龄组中的EBV相关T细胞和NK细胞淋巴增殖性疾病(LPD)包括全身性疾病、慢性活动性EBV感染(CAEBV)和儿童期全身性EBV+ T细胞淋巴瘤。种痘样水疱(HV)样LPD是CAEBV的主要皮肤形式,包括以前称为HV和HV样淋巴瘤(HVLL)的病变。所有T/NK细胞-EBV相关疾病在亚洲人、中美洲和南美洲以及墨西哥的土著人群中发生频率较高。在B细胞EBV相关的LPD中,WHO引入了两个主要变化。以前指定的老年人EBV阳性弥漫性大B细胞淋巴瘤(EBVDLB CL)已改为EBV-DLBCL,并以“未另行说明”作为修饰语(NOS)。WHO系统的一个新的补充是最近发现的EBV+皮肤粘膜溃疡,涉及皮肤和粘膜相关部位。
This article will focus on the cutaneous lymphoproliferative disorders associated with EBV, with an emphasis on the upcoming changes in the revised 4th Edition of the WHO classification of tumors of the hematopoietic system, many of which deal with cutaneous disorders derived from NK-cells or T-cells. Extranodal NK/T-cell lymphoma usually presents in the upper aerodigestive tract, but can involve the skin secondarily. EBV-associated T- and NK-cell lymphoproliferative disorders (LPD) in the pediatric age group include the systemic diseases, chronic active EBV infection (CAEBV) and systemic EBV+ T-cell lymphoma of childhood. Hydroa vacciniforme (HV)-like LPD is a primarily cutaneous form of CAEBV and encompasses the lesions previously referred to as HV and HV-like lymphoma (HVLL). All the T/NK-cell-EBV-associated diseases occur with higher frequency in Asians, and indigenous populations from Central and South America and Mexico. Among the B-cell EBV-associated LPD two major changes have been introduced in the WHO. The previously designated EBV-positive diffuse large B-cell lymphoma (EBVDLBCL) of the elderly, has been changed to EBV-DLBCL with ‘not otherwise specified’ as a modifier (NOS). A new addition to the WHO system is the more recently identified EBV+ mucocutaneous ulcer, which involves skin and mucosal-associated sites.
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