Kim I S, Park C K, Kim H, Lee Y Y, Ahn M J, Lee W S
Department of Internal Medicine, Hanyang University Hospital, Seoul, Korea.
J Korean Med Sci. 1997 Oct;12(5):447-51. doi: 10.3346/jkms.1997.12.5.447.
Virus associated hemophagocytic syndrome (VAHS) are a heterogeneous group of disorders in which viral infection is associated with a proliferation of hemophagocytic histiocytes through the reticuloendothelial system. We report the case of a 21-year-old Korean man who presented to us with high fever, marked hepatosplenomegaly, severe hepatic dysfunction, coagulopathy, pancytopenia and marked panhypogammaglobulinemia. Bone marrow aspiration and biopsy showed histiocytes proliferation with active phagocytosis of red cells and neutrophils. Primary Epstein-Barr (EB) viral infection at presentation was confirmed by the presence of IgM antibody to viral capsid antigen (VCA) with absence of antibody to EB viral nuclear antigen (EBNA). A liver biopsy performed one month after the presentation showed erythrophagocytic histiocytes within the sinusoids. EB virus was demonstrated in the liver biopsy tissue by DNA PCR method, and EBER mRNA in situ hybridization.
病毒相关性噬血细胞综合征（VAHS）是一组异质性疾病，其中病毒感染与噬血细胞组织细胞通过网状内皮系统增殖有关。我们报告了一例21岁韩国男性病例，该患者出现高热、明显肝脾肿大、严重肝功能障碍、凝血病、全血细胞减少和明显的全低丙种球蛋白血症。骨髓穿刺和活检显示组织细胞增殖，并伴有红细胞和中性粒细胞的活跃吞噬。通过病毒衣壳抗原（VCA）IgM抗体的存在以及EB病毒核抗原（EBNA）抗体的缺失，证实了初诊时原发性爱泼斯坦-巴尔（EB）病毒感染。初诊后一个月进行的肝脏活检显示，肝血窦内有噬红细胞组织细胞。通过DNA PCR方法和EBER mRNA原位杂交在肝脏活检组织中检测到EB病毒。
