4例EB病毒感染相关急性肝功能衰竭患儿的临床特点分析

Clinical features of children with Epstein-Barr virus-related acute liver failure: an analysis of four cases

摘要:

4例EB病毒感染相关急性肝功能衰竭患儿中男2例、女2例，年龄10（8.5~44）个月。3例诊断为传染性单核细胞增多症（IM），其中2例符合噬血细胞淋巴组织细胞增生症（HLH）诊断标准；1例诊断为EBV既往感染。4例患儿的血EBV-DNA载量均阳性。4例患儿入院时均有发热、肝大、黄疸，3例患儿具有脾大、腹水或者呕吐症状，2例患儿有颈淋巴结大、皮疹或胸水，1例患儿出现消化道出血或者2期肝性脑病。4例患儿的异型淋巴细胞计数均 < 10%，仅1例出现白细胞升高和血小板减少；4例患儿的转氨酶10~100倍升高、以直接胆红素为主的总胆红素3~5倍升高、乳酸脱氢酶数10倍升高、凝血酶原时间显著延长。4例患儿均静脉给予阿昔洛韦或更昔洛韦抗病毒、促肝细胞生长因子促进肝细胞生长以及激素减轻炎症反应等治疗；2例加用血浆置换治疗，其中1例联合连续性静脉-静脉血液透析滤过治疗；2例HLH患儿按照HLH 2004方案化疗。3例存活，1例HLH因多脏器功能衰竭死亡。EB病毒感染可以引起儿童急性肝功能衰竭，早期给予包括血液净化治疗在内的综合治疗手段可能对预后有益。

Abstract:

A retrospective analysis was performed for the clinical data of four children with Epstein-Barr virus (EBV)-related acute liver failure. There were two boys and two girls with a median age of 10 months (range 8.5-44 months). Of the four children, three were diagnosed with infectious mononucleosis (IM), among whom two met the diagnostic criteria of hemophagocytic lymphohistiocytosis (HLH), and one was diagnosed with past EBV infection. All the children had positive EBV DNA in blood and all had pyrexia, hepatomegaly, and jaundice on admission. Three children had the symptom of splenomegaly, ascites, or vomiting. Two children had enlargement of cervical lymph nodes, skin rash, or pleural effusion. One child had gastrointestinal bleeding or stage 2 hepatic encephalopathy. All the children had an abnormal lymphocyte count of < 10%, and only one child had leukocytosis and thrombocytopenia. Among the four children, alanine aminotransferase level increased by 10-100 times; total bilirubin level increased by 3-5 times; lactate dehydrogenase level increased by many 10 times; prothrombin time prolonged significantly. All the children were given antiviral therapy with intravenously injected acyclovir or ganciclovir, as well as hepatocyte growth factor to promote hepatocyte growth and hormone to alleviate inflammatory response. Two children were given plasma exchange in addition, among whom one was given the combination of continuous venovenous hemodiafiltration. Two children with HLH were given chemotherapy according to the HLH-2004 regimen. Three children survived, and one child with HLH died of multiple organ failure. It is concluded that EBV infection can cause acute liver failure and that early use of multimodality therapy including blood purification may be beneficial for prognosis in these children.