Raphael M
Service d'hématologie biologique. Hôpital Avicenne, Bobigny.
Bull Acad Natl Med. 1997 Jun-Jul;181(6):999-1007; discussion 1007-8.
The EBV plays a major role in the development of lymphoproliferative disorders in immunosuppressed patients. After organ transplantation most of lymphoproliferative disorders associated with EBV are polymorphic, with various expression of clonality. The pattern of EBV latency genes expression is rather the same as in lymphoblastoid cells lines and the EBV infected cells strongly expressed activation and adhesion molecules in most cases. In AIDS-related lymphomas the frequency of EBV as well as the expression of latency genes are related to the localization and to the histological subtypes. While EBV is observed in 30 to 50% of cases of Burkitt's lymphomas occurring the early stage of AIDS, its association in primary brain lymphomas and immunoblastic lymphomas developed in the late stage is observed in nearly all cases as well as in Hodgkin's disease. In primary brain lymphomas, the high expression of LMP-1 protein is correlated to the expression of BCL2 oncoprotein suggesting a transactivation of bcl2 by LMP-1 as it was reported in vitro. In non overt immunosuppressed patients the role of EBV is less clearly established, particularly in Burkitt's lymphoma where EBV is now considered as a cofactor. In B-cell lymphoma EBV is detected in about 5% of cases except in peculiar situations such as in lymphoma occurring in pleural cavity after longstanding pleural chronic inflammation and in Richter's syndrome with Reed-Sternberg-like cells. In peripheral T-cell lymphomas, EBV is observed in about 25% of cases, but its frequency varies with the histology and the localisation. EBV is present in nearly all cases of angio-immunoblastic type and in the nasal lymphoid proliferations developed from the NK cells. Detected in 30 to 80% in the Reed-Sternberg cells of Hodgkin's disease cases, the pathogenic significance of EBV remains to be determined in this disease.
EB病毒在免疫抑制患者淋巴增殖性疾病的发生中起主要作用。器官移植后，大多数与EB病毒相关的淋巴增殖性疾病是多形性的，具有不同的克隆性表达。EB病毒潜伏基因的表达模式与淋巴母细胞系中的模式相当，并且在大多数情况下，EB病毒感染的细胞强烈表达激活分子和黏附分子。在艾滋病相关淋巴瘤中，EB病毒的频率以及潜伏基因的表达与定位和组织学亚型有关。虽然在艾滋病早期发生的伯基特淋巴瘤病例中有30%至50%可观察到EB病毒，但在晚期发生的原发性脑淋巴瘤和免疫母细胞淋巴瘤中，几乎所有病例以及霍奇金病中都可观察到其关联。在原发性脑淋巴瘤中，LMP-1蛋白的高表达与BCL2癌蛋白的表达相关，提示LMP-1如体外报道的那样对bcl2进行反式激活。在非明显免疫抑制的患者中，EB病毒的作用尚不太明确，特别是在伯基特淋巴瘤中，EB病毒现在被认为是一种辅助因子。在B细胞淋巴瘤中，约5%的病例可检测到EB病毒，但在特殊情况下除外，如长期胸膜慢性炎症后胸腔内发生的淋巴瘤以及伴有里德-斯腾伯格样细胞的里氏综合征。在外周T细胞淋巴瘤中，约25%的病例可观察到EB病毒，但其频率因组织学和定位而异。几乎所有血管免疫母细胞型病例以及由NK细胞发展而来的鼻淋巴增殖中都存在EB病毒。在霍奇金病病例的里德-斯腾伯格细胞中，EB病毒的检出率为30%至80%，其在该疾病中的致病意义仍有待确定。
