Department of Hematopathology, University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Blood. 2013 Jul 18;122(3):328-40. doi: 10.1182/blood-2013-03-489708. Epub 2013 May 6.
Epstein-Barr virus (EBV) positive diffuse large B-cell lymphoma (DLBCL) of the elderly, initially described in 2003, is a provisional entity in the 2008 World Health Organization classification system and is defined as an EBV-positive monoclonal large B-cell proliferation that occurs in patients >50 years of age and in whom there is no known immunodeficiency or history of lymphoma. These tumors are more common in Asia but also occur in North America and Europe at a low frequency. These neoplasms exhibit a morphologic continuum, from polymorphous to monomorphous, but morphologic features do not correlate with prognosis as all patients have a clinically aggressive course. Most EBV-positive DLBCL of the elderly patients have an activated B-cell immunophenotype and are characterized by prominent nuclear factor-κB activation. Cytogenetic complexity is usually low. In this review, we comprehensively delineate the data emerging from analyses of EBV latency program, microRNA-mediated EBV viral oncogenesis, functional genomics of EBV and its biology, and differential diagnosis challenge for EBV-positive DLBCL of the elderly. It is hoped that the improved understanding of these tumors will lead to the development of novel therapeutic approaches, enhance the effectiveness of clinical trials, and improve prognosis.
老年 EBV 阳性弥漫性大 B 细胞淋巴瘤（DLBCL）最初于 2003 年描述，是 2008 年世界卫生组织分类系统中的一个暂定实体，定义为发生在>50 岁且无已知免疫缺陷或淋巴瘤病史的患者中的 EBV 阳性单克隆大 B 细胞增殖。这些肿瘤在亚洲更为常见，但在北美和欧洲也以低频率发生。这些肿瘤表现出形态学连续体，从多形性到单形性，但形态学特征与预后无关，因为所有患者均具有临床侵袭性病程。大多数老年 EBV 阳性 DLBCL 患者具有活化 B 细胞免疫表型，并以显著的核因子-κB 激活为特征。细胞遗传学复杂性通常较低。在这篇综述中，我们全面描述了 EBV 潜伏期程序、miRNA 介导的 EBV 病毒致癌作用、EBV 的功能基因组及其生物学以及老年 EBV 阳性 DLBCL 的鉴别诊断挑战方面的分析结果。希望对这些肿瘤的深入了解将导致新的治疗方法的发展，提高临床试验的效果，并改善预后。
