Age-related Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorders: comparison with EBV-positive classic Hodgkin lymphoma in elderly patients
Age-related Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorders: comparison with EBV-positive classic Hodgkin lymphoma in elderly patients
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DOI：
10.1182/blood-2008-06-164806
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发表时间：
2009-03-19
期刊：
影响因子：
20.3
通讯作者：
Nakamura, Shigeo
中科院分区：
文献类型：
--
作者：
Asano, Naoko;Yamamoto, Kazuhito;Nakamura, Shigeo
年龄相关性EB病毒相关性B细胞淋巴增生性疾病(AEBVLPD)是一种以EB病毒相关性大B细胞淋巴瘤为特征的疾病组，在老年人中不会导致免疫缺陷。在近三分之一的病例中，EBVLPD是一种具有丰富反应细胞成分的多形性亚型，在形态上与经典霍奇金淋巴瘤(CHL)相似。本研究的目的是阐明50岁或以上患者中aEBVLPD多态亚型(n=34)和EBV+CHL亚型(n=108)的临床病理差异。结果表明，aEBVLPD与侵袭性临床参数的关系比CHL更密切，发病年龄较高(71vs63岁)，男性占优势(1.4vs3.3)，皮肤(18%vs2%)、胃肠道(15%vs4%)和肺部(12%vs2%)的受累率更高。AEBVLPD的组织病理学特征是地理坏死率较高，背景淋巴细胞中细胞毒性T细胞增加(>；30%)，CD20和EBNA2阳性率较高，CD15表达缺失。正如临床资料所预测的那样，aEBVLPD的预后明显差于EBV+CHL(P
Age-related Epstein-Barr virus-associated B-cell lymphoproliferative disorder (aEBVLPD) is a disease group characterized by EBV-associated large B-cell lymphoma in the elderly without predisposing immunodeficiency. In nearly one-third of cases, aEBVLPD occurs as a polymorphous subtype with reactive cell-rich components, bearing a morphologic similarity to classic Hodgkin lymphoma (cHL). The aim of this study was to clarify clinicopathologic differences between the polymorphic subtype of aEBVLPD (n = 34) and EBV+ cHL (n = 108) in patients aged 50 years or older. Results showed that aEBVLPD was more closely associated with aggressive clinical parameters than cHL, with a higher age at onset (71 vs 63 years); lower male predominance (male-female ratio, 1.4 vs 3.3); and a higher rate of involvement of the skin (18% vs 2%), gastrointestinal tract (15% vs 4%), and lung (12% vs 2%). aEBVLPD was histopathologically characterized by a higher ratio of geographic necrosis, greater increase (>30%) in cytotoxic T cells among background lymphocytes, higher positivity for CD20 and EBNA2, and absence of CD15 expression. As predicted by the clinical profile, aEBVLPD had a significantly poorer prognosis than EBV+ cHL (P