Okano M, Thiele G M, Gross T G, Davis J R, Purtilo D T
Department of Pathology, University of Nebraska Medical Center, Omaha 68198-3135.
J Med Virol. 1990 Sep;32(1):47-52. doi: 10.1002/jmv.1890320108.
Epstein-Barr virus (EBV) is often associated with lethal lymphoproliferative diseases in immunologically compromised individuals. Recently, we have studied a 20-month-old boy with X-linked lymphoproliferative disease (XLP) who had succumbed to infectious mononucleosis (IM) complicated by fulminant hepatitis and virus-associated hemophagocytic syndrome following EBV infection. EBV genomes were detected in peripheral blood lymphocytes (PBL), cervical and mesenteric lymph nodes, liver, spleen, thymus, and bone marrow. According to restriction endonuclease analyses, the EBV-DNA pattern was similar in all samples except for the EBV-DNA from the bone marrow. Additionally, circular EBV-DNA (suggesting a latent infection) predominated in spontaneously established lymphoblastoid cell lines (LCLs) derived from both the lymph node and cord lymphocytes co-cultured with PBL. In contrast, both circular and linear EBV-DNA (suggesting a lytic infection) were noted in spontaneously established LCLs derived from his PBL. Furthermore, LCLs derived from both the lymph node and cord lymphocytes co-cultured with PBL expressed fewer reactive cells for early antigen (EA) and viral capsid antigen (VCA) than spontaneous LCLs from his PBL, thus providing evidence for different B cellular susceptibility to EBV infection in this patient with XLP. Finally, defective EBV-specific cytotoxic T cell activity was observed in this patient. Latent EBV infected cells may easily escape immunosurveillance by the host. These findings may explain the fatal course of EBV infection in this patient.
爱泼斯坦-巴尔病毒（EBV）常与免疫功能低下个体的致死性淋巴增殖性疾病相关。最近，我们研究了一名患有X连锁淋巴增殖性疾病（XLP）的20个月大男孩，他在感染EBV后死于传染性单核细胞增多症（IM），并发暴发性肝炎和病毒相关噬血细胞综合征。在其外周血淋巴细胞（PBL）、颈部和肠系膜淋巴结、肝脏、脾脏、胸腺和骨髓中检测到了EBV基因组。根据限制性内切酶分析，除骨髓中的EBV-DNA外，所有样本中的EBV-DNA模式相似。此外，在与PBL共培养的淋巴结和脐带淋巴细胞自发建立的淋巴母细胞系（LCL）中，环状EBV-DNA（提示潜伏感染）占主导。相比之下，在源自其PBL的自发建立的LCL中，同时检测到环状和线性EBV-DNA（提示裂解感染）。此外，与PBL共培养的淋巴结和脐带淋巴细胞自发建立的LCL中，早期抗原（EA）和病毒衣壳抗原（VCA）的反应性细胞比源自其PBL的自发LCL少，从而为该XLP患者中不同B细胞对EBV感染的易感性提供了证据。最后，在该患者中观察到EBV特异性细胞毒性T细胞活性缺陷。潜伏感染的EBV细胞可能很容易逃避宿主的免疫监视。这些发现可能解释了该患者EBV感染的致命病程。
