Clinicopathologic and molecular biological study of senile/age-related EBV-associated B-cell

恶性淋巴瘤在临床、生物学和地方性特征上具有广泛的异质性。EB病毒（Epstein-Barr virus，EBV）与一些恶性淋巴瘤相关，包括Burkitt淋巴瘤、霍奇金淋巴瘤、免疫缺陷相关淋巴组织增生性疾病（Immunodeficiency-associated lymphoproliferative disorders，LPD）和部分弥漫性大B细胞淋巴瘤。在2003年，我们报道了22例名为老年EB病毒相关B细胞淋巴增生性疾病（B-LPD）的病例，分为多态性和大细胞淋巴瘤（LCL）亚型，发生于年龄>60岁的老年患者，无易患免疫缺陷（Am J Surg Pathol 2003;27：16）。肿瘤细胞表达CD 20和/或CD 79 a，原位杂交显示它们与EBV相关。所有病例均检测到LMP 1，7例（32%）检测到EBNA 2，分别表示潜伏期II和III状态。这种疾病在形态学上常被不同数量的HRS样巨细胞所包围，并提示与免疫球蛋白相关。 ...更多信息 为了进一步阐明这种新认识的疾病实体的临床病理特征和谱，我们随后在1990年1月至2003年12月期间检索了134例EBV阳性B细胞LPD，这些病例没有潜在的免疫缺陷相关疾病，作为多机构研究，000例弥漫性大B细胞淋巴瘤及相关疾病。目前正在对它们的总体临床病理特征进行详细的研究。简而言之，这一更大的系列研究显示，126例（94%）患者年龄超过50岁，提供了额外的证据，即没有诱发免疫缺陷的EBV阳性LPD主要发生在老年患者中。然而，发现8例患者年龄小于50岁，尽管其与慢性活动性EBV感染和/或原发性免疫疾病的关系仍有待充分阐明。考虑到这些罕见病例，“年龄相关”一词可能比“老年”一词更适合于进一步了解无诱发免疫缺陷的EBV阳性B细胞LPD的总体年龄分布。少

Malignant lymphoma is widely heterogeneous in its clinical, biologic, and endemic properties. Epstein-Barr virus (EBV) is associated with some disease entities of malignant lymphomas, including Burkitt lymphoma, Hodgkin lymphoma, immunodeficiency-associated lymphoproliferative disorders (LPDs) and a part of diffuse large B-cell lymphoma. In 2003, we reported 22 cases named as senile EBV-associated B-cell lymphoproliferative disorders (B-LPDs), which were divided into polymorphic and large-cell lymphoma (LCL) subtypes, arising in elderly patients aged >60 years without predisposing immunodeficiencies (Am J Surg Pathol 2003;27:16). Tumor cells expressed CD20 and/or CD79a, and in situ hybridization showed them to be associated with EBV. LMP1 was detected in all cases and EBNA2 in seven (32%), indicating latency II and III status respectively. This disease was often morphologically accompanid by a varying number of HRS-like giant cells, and suggested to have a relationship with an immunolo … More gical deterioration derived from the aging process.In order to further address the clinicopathologic profiles and spectrum for this newly-recognized disease entity, we subsequently searched 134 cases of the EBV-positive B-cell LPDs without underlying immunodeficiency-related diseases during the period from January 1990 and December 2003 as the multi-institutional study, which have been retrieved from 2,000 patients with diffuse large B-cell lymphoma and the related diseases. Their overall clinicopthologic profiles are now under investigation in details. In brief, this larger series revealed that 126 (94%) of the patients are more than 50 years old of age, providing an additional evidence that EBV-positive LPDs without predisposing immunodeficiency mainly occurs in elderly patients. However, 8 patients were found to be less than 50 years old of age, although it remains to be adequately elucidated about their relationship with chronic active EBV infection and/or primary immune disorders. In taking these rare cases into the consideration, the term of "age-related" may be more appropriate than that of "senile" for further understanding the overall age distribution of EBV-positive B-cell LPDs without predisposing immunodeficiency. Less