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与年龄相关的 EBV 相关 B 细胞淋巴增生性疾病:一种新认识的临床病理实体的诊断方法。

Age-related EBV-associated B-cell lymphoproliferative disorders: diagnostic approach to a newly recognized clinicopathological entity.

机构信息

Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan.

出版信息

Pathol Int. 2009 Dec;59(12):835-43. doi: 10.1111/j.1440-1827.2009.02466.x.

DOI:10.1111/j.1440-1827.2009.02466.x
PMID:20021607
Abstract

EBV is prevalent among healthy individuals, and is implicated in numerous reactive and neoplastic processes in the immune system. The authors originally identified a series of senile or age-related EBV-associated B-cell lymphoproliferative disorders (LPD) bearing a resemblance to immunodeficiency-associated ones, which may be associated with immune senescence in the elderly and which are now incorporated into the 2008 World Health Organization lymphoma classification as EBV-positive diffuse large B-cell lymphoma (DLBCL) of the elderly. This newly described disease is pathologically characterized by a proliferation of atypical large B cells including Reed-Sternberg-like cells with reactive components, which pose a diagnostic problem for pathologists. Clinically, this disease may present with lymphadenopathy, and is often extranodal, frequently involving the skin, gastrointestinal tract, or lung. Onset is usually after the age of 50; the median patient age is 70-79 years, and incidence continues to increase with age, providing additional support to the nosological term of EBV+ DLBCL of the elderly. These patients have a worse prognosis than those with EBV-negative DLBCL or EBV+ classical Hodgkin lymphoma (CHL). The aim of the present review was to summarize the clinicopathological profile of age-related EBV+ LPD and EBV+ Hodgkin lymphoma to facilitate diagnostic approach.

摘要

EBV 在健康个体中普遍存在,并与免疫系统中的许多反应性和肿瘤性过程有关。作者最初鉴定了一系列类似于与免疫缺陷相关的与衰老相关的 EBV 相关 B 细胞淋巴增生性疾病(LPD),这些疾病可能与老年人的免疫衰老有关,现在已被纳入 2008 年世界卫生组织淋巴瘤分类,作为 EBV 阳性弥漫性大 B 细胞淋巴瘤(DLBCL)的老年人。这种新描述的疾病在病理学上表现为包括 Reed-Sternberg 样细胞在内的异型大 B 细胞的增殖,伴有反应性成分,这给病理学家带来了诊断问题。临床上,这种疾病可能表现为淋巴结病,通常为结外,常累及皮肤、胃肠道或肺部。发病通常在 50 岁以后;中位患者年龄为 70-79 岁,发病率随着年龄的增长而持续增加,为老年 EBV+ DLBCL 的病名提供了更多支持。与 EBV 阴性 DLBCL 或 EBV+经典霍奇金淋巴瘤(CHL)相比,这些患者的预后更差。本综述的目的是总结与年龄相关的 EBV+LPD 和 EBV+霍奇金淋巴瘤的临床病理特征,以促进诊断方法。

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