Nakagawa A, Ito M, Iwaki T, Yatabe Y, Asai J, Hayashi K
Department of Pathology, Nagoya University Hospital, Japan.
Am J Clin Pathol. 1996 Jun;105(6):733-6. doi: 10.1093/ajcp/105.6.733.
Chronic active Epstein-Barr virus infection (CAEBV) is a nonfamilial syndrome that shows a specific immunodeficiency for the Epstein-Barr virus (EBV). Prolonged fever, hepatosplenomegaly, lymphadenopathy, and liver dysfunction were seen in CAEBV, but cardiac complications are rare. An autopsy case of CAEBV with giant coronary aneurysms and aortic aneurysms is reported. The patient was a 5-year-old Japanese girl. At autopsy, the heart weighed 110 g, and bilateral coronary aneurysms were found. Microscopic studies revealed lymphoid vasculitis of coronary arteries, coronary venules, and aortic arteries. Immunohistochemically, infiltrating small lymphocytes were positive for CD3, CD45RO(UCHL-1), CD43(DF-T1). The presence of EBV in most of these T lymphocytes was proven by in situ hybridization using an EBV-encoded RNA-1 (EBER1) probe. To the best of the author's knowledge, pathology of aneurysms caused by lymphoid vasculitis in CAEBV has not been reported until now.
慢性活动性EB病毒感染（CAEBV）是一种非家族性综合征，表现为对EB病毒（EBV）的特异性免疫缺陷。CAEBV患者可见长期发热、肝脾肿大、淋巴结病和肝功能障碍，但心脏并发症罕见。本文报道1例CAEBV尸检病例，该患者合并巨大冠状动脉瘤和主动脉瘤。患者为一名5岁日本女孩。尸检时，心脏重量为110g，发现双侧冠状动脉瘤。显微镜检查显示冠状动脉、冠状小静脉和主动脉存在淋巴细胞性血管炎。免疫组化显示，浸润的小淋巴细胞CD3、CD45RO（UCHL-1）、CD43（DF-T1）呈阳性。使用EBV编码RNA-1（EBER1）探针进行原位杂交证实，这些T淋巴细胞中的大多数存在EBV。据作者所知，此前尚未报道过CAEBV中由淋巴细胞性血管炎引起的动脉瘤的病理学情况。
