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Epstein-Barr病毒感染合併血球吞噬症候群：一病例報告
Epstein-Barr Virus-associated Hemophagocytic Syndrome: a Case Report
摘要
病毒感染合併血球吞噬症候群（virus-associated hemophagocytic syndrome）的診斷是在1979年由Risdall等人提出，它描述一種由病毒感染造成全身性良性組織球之增生合併嚴重之血球吞噬現象。臨床表徵有週邊全部血球減少、高燒、肝細胞功能異常、肝脾腫大及凝血功能異常。此疾病和一群感染原有關，包括病毒、細菌、黴菌及寄生蟲。我們提出一位十四歲女孩在入 院時有高燒、喉嚨疼痛、頭痛、倦怠、淋巴結腫大、黃疸、肝脾腫大、肝功能異常及全部血球減少的臨床症狀。骨髓穿刺檢查顯示有明顯的巨噬細胞吞噬血球現象。血清抗體檢查顯示高效價抗Epstein-Barr病毒（EBV）莢膜抗原IgM抗體（Anti-EBV-VCA IgM Ab）及低效價抗EBV核抗原抗體（Anti-EBNA Ab），證實病人是原發性的EBV感染。我們以靜脈注射免疫球蛋白G（每天每公斤體重1公克，共二天）及類固醇治療病人，病人的治療反應非常好。此項結果告訴我們在EBV感染造成血球吞噬症候群的發病早期可嘗試使用靜脈注射免疫球蛋白G及類固醇來治療。
並列摘要
The term ”virus-associated hemophagocytic syndrome” (VAHS) was introduced by Risdall et al in 1979 to describe a disorder characterized by a benign generalized histiocytic proliferation with marked hemophagocytosis associated with systemic viral infection. Clinical manifestation include high fever, pancytopenia, hepatocellular dysfunction, hepatosplenomegaly and coagulopathy. The disorder has been associated with a wide variety of infectious agents, including viruses, bacteria, fungi and parasites. A 14-year-old girl was admitted with high fever, sore-throat, headache, fatigue, jaundice, lymphadenopathy, hepatosplenomegaly, liver dysfunction and pancytopenia. Bone marrow aspiration showed apparent hemophagocytosis by macrophages. Primary Epstein-Barr virus (EBV) infection was confirmed by high titers of IgM antibody to viral capsid antigen (VCA) with low titers of antibody to EBV nuclear antiger (EBNA). Intravenous immune globulin G (1 g/kg/day for 2 days) and prednisolone therapy was administered. The patient responded very well. Immune globulin G and steroid may consider as a treatment in the early stage of this syndrome.