Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Michigan Health System, 3912 Taubman Center, 1500 E. Medical Center Drive, SPC 5362, Ann Arbor, MI, 48109-0362, USA.
Dig Dis Sci. 2014 Jul;59(7):1630-7. doi: 10.1007/s10620-014-3029-2. Epub 2014 Jan 25.
Acute liver failure (ALF) is a rare clinical syndrome associated with a high case fatality rate. Asymptomatic primary infection with Epstein-Barr virus (EBV) is common in the general population while acute hepatitis and jaundice are much less common and ALF has been rarely reported. We reviewed the presenting features as well as clinical outcomes amongst consecutive adults with EBV-related ALF.
Amongst the 1,887 adult ALF patients enrolled into the US ALF Study Group from January 1998 to February 2012, there were four patients (0.21 %) with EBV-related ALF. Diagnostic criteria for acute EBV infection included compatible serologies and/or the detection of EBV-encoded RNA (EBER) in liver tissue.
Median patient age was 30 years (range 18-44); 75 % were male, and only 25 % were immunosuppressed. The median presenting ALT was 504 IU/mL (range 156-4,920), median Alk P was 431 (range 136-1,009), and median bilirubin was 17 mg/dL (range 13-22.1). Liver biopsy findings ranged from cholestasis to submassive necrosis with EBER + staining in two of the three samples tested. Although all of the patients were treated with an antiviral agent, two died of ALF, one underwent liver transplantation (LT) and one survived with supportive care and is well at 5 years. A review of the literature identified four additional LT recipients with favorable long-term outcomes.
Primary EBV infection accounts for <1 % of consecutive adult ALF cases but is associated with a high case fatality rate. LT is associated with favorable short- and long-term outcomes.
急性肝衰竭（ALF）是一种罕见的临床综合征，病死率高。人群中无症状的原发性 EBV 感染很常见，而急性肝炎和黄疸则较少见，ALF 更是罕见报道。我们回顾了连续成人 EBV 相关 ALF 的临床表现和临床结局。
1998 年 1 月至 2012 年 2 月，美国 ALF 研究组纳入了 1887 例成人 ALF 患者，其中有 4 例（0.21%）为 EBV 相关 ALF。急性 EBV 感染的诊断标准包括一致的血清学和/或肝组织中 EBV 编码 RNA（EBER）的检测。
中位患者年龄 30 岁（范围 18-44）；75%为男性，仅有 25%为免疫抑制。中位 ALT 为 504IU/ml（范围 156-4920），中位碱性磷酸酶为 431（范围 136-1009），中位胆红素为 17mg/dl（范围 13-22.1）。肝活检结果从胆汁淤积到亚大块坏死，在 3 个检测样本中的 2 个样本中 EBER+染色阳性。尽管所有患者均接受抗病毒药物治疗，但有 2 例死于 ALF，1 例接受了肝移植（LT），1 例接受支持治疗后存活，5 年后情况良好。文献复习发现另外 4 例 LT 受者预后良好。
原发性 EBV 感染占连续成人 ALF 病例的<1%，但病死率高。LT 与良好的短期和长期预后相关。
