Kitazawa Yasuhide, Saito Fukuki, Nomura Shosaku, Ishii Kazuyoshi, Kadota Eiji
Department of Emergency and Critical Care Medicine, Kansai Medical University Hirakata Hospital, Hirakata-city, Osaka, Japan.
Clin Appl Thromb Hemost. 2007 Jul;13(3):323-8. doi: 10.1177/1076029607302570.
The Epstein-Barr virus (EBV) infection is known to result in infectious mononucleosis, hemophagocytic syndrome, chronic active EBV infection, and lymphoma. Among them, hemophagocytic syndrome sometimes causes thrombocytopenia, which is often life threatening because of its hemorrhagic complications such as gastrointestinal bleeding and pulmonary alveolar hemorrhage. A young adult case of critical hemophagocytic syndrome after primary EBV infection is presented. Chemotherapy was performed using methyl prednisolone succinate, prednisolone, cyclosporin A, and 20 mg/kg of cyclophosphamide. The patient received intensive care, including plasma exchange for hepatic failure, extracorporeal membrane oxygenation for acute respiratory failure, and splenectomy for hemophagocytosis; however, the patient died of multiple organ failure, including fulminant hepatic failure. The pathologic examination of the resected specimen demonstrated infiltrated macrophages containing many phagocytosed erythrocytes. Further immunopathologic examination of these cells showed that the histiocyte markers were positive, whereas the T-cell marker was negative. In view of these findings, definite diagnosis of EBV-related hemophagocytic lymphohistiocytosis could not be made at that time. The immunohistologic examinations on the liver necropsy specimen provided the evidences suggesting the morbid activation of the hepatic stellate macrophage by EBV-infected T/NK cells and subsequent apoptosis induction of the liver cells through the Fas ligand pathway.
已知爱泼斯坦-巴尔病毒（EBV）感染会导致传染性单核细胞增多症、噬血细胞综合征、慢性活动性EBV感染和淋巴瘤。其中，噬血细胞综合征有时会导致血小板减少，由于其诸如胃肠道出血和肺泡出血等出血性并发症，往往会危及生命。本文介绍了一例原发性EBV感染后发生严重噬血细胞综合征的年轻成人病例。使用琥珀酸甲泼尼龙、泼尼松龙、环孢素A和20mg/kg环磷酰胺进行化疗。患者接受了重症监护，包括针对肝衰竭的血浆置换、针对急性呼吸衰竭的体外膜肺氧合以及针对噬血细胞增多症的脾切除术；然而，患者死于多器官衰竭，包括暴发性肝衰竭。对切除标本的病理检查显示浸润的巨噬细胞含有许多吞噬的红细胞。对这些细胞进一步的免疫病理检查表明组织细胞标志物呈阳性，而T细胞标志物呈阴性。鉴于这些发现，当时无法对EBV相关噬血细胞性淋巴组织细胞增生症做出明确诊断。对肝脏尸检标本的免疫组织学检查提供了证据，表明EBV感染的T/NK细胞使肝星状巨噬细胞发生病态激活，并随后通过Fas配体途径诱导肝细胞凋亡。
