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

老年/年龄相关 EBV 相关 B 细胞的临床病理学和分子生物学研究

基本信息

批准号：
16590301
负责人：
NAKAMURA Shigeo
金额：
$ 2.3万
依托单位：
Nagoya University (2005)Aichi Cancer Center Research Institute (2004)
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (C)
财政年份：
2004
资助国家：
日本
起止时间：
2004 至 2005
项目状态：
已结题

项目摘要

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

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。考虑到这些罕见的病例，“年龄相关”的术语可能比“老年”更适合于进一步了解EBV阳性B细胞LPD的总体年龄分布，而没有诱发免疫缺陷。少