慢性活动性EB病毒感染临床分析

Clinical Analysis of Chronic Active Epstein-Barr Virus Infection

目的通过总结、分析慢性活动性EB病毒感染(CAEBV)患者的临床资料,以提高对CAEBV的诊断、治疗的认识。方法回顾性分析2008年10月-2013年1月诊治的9例CAEBV患者的临床症状特点,以及实验室检查、病原学相关检查、影像学和病理学检查结果等,并评估其对药物治疗的反应和随访、预后表现。结果 CAEBV临床症状缺乏特异性,主要表现为发热、肝脾和淋巴结肿大,其他尚有乏力、恶心、皮疹、黄疸等。辅助检查的异常发现包括:贫血、白细胞降低、中性粒细胞降低、血小板减少、乳酸脱氢酶及羟丁酸脱氢酶升高等肝功能异常及胸部影像学的异常表现等。病原学相关检查结果:6例患者抗EB病毒衣壳抗原IgA抗体升高,8例EB病毒早期抗原IgG为阳性,实时定量聚合酶链反应检测外周血EB病毒DNA载量(中位数)为3.07×105 copies/mL。9例患者中6例死亡,其中1例死于颅内出血,1例死于多脏器功能衰竭,1例死于噬血细胞综合征,l例死于肺部感染;4例患者进展为淋巴瘤,在抗肿瘤治疗过程中1例死于肝功能衰竭,1例死于严重感染。结论 CAEBV临床表现多样且缺乏特异性,常伴有各系统严重并发症,部分患者可进展为淋巴细胞增殖性疾病,预后差,病死率高,应引起临床重视。

Objective To improve the understanding of the diagnosis and therapy of chronic active Epstein- Barr virus （CAEBV） infection. Methods Data of 9 cases of CAEBV infection diagnosed between October 2008 and January 2013 were analyzed retrospectively, including clinical and auxiliary examination results, pathological data, especially EB virus （EBV） antibodies and DNA in peripheral blood mononuclear cells （PBMC） and infected tissue, and follow-up information. Results The major manifestations of the 9 patients were fever, splenomegaly, hepatomegaly, lymphadenopathy, and others, including general fatigue, nausea, skin rash, jaundice, and so on. The abnormalities of auxiliary examination were as follows： anemia, leucopenia, neutropenia, thrombocytopenia, elevated LDH and HBDH levels, liver dysfunction and abnormal chest CT findings. EBV serologic tests revealed high IgA antibody levels against EB viral capsid antigen （VCA） in 6 patients, and 8 patients had positive IgG antibody levels against early D antigen （EAD）. The mean load of EBV-DNA detected by real time polymerase chain reaction （PCR） in the PBMC was 3.07×10^5 copies/mL. Six of the nine patients presented a poor clinical course. One of them died of intracranial hemorrhage, one of them died of multiple organ failure, one of them died of EBV-associated hemophagocytic syndrome, and one of them died of severe pulmonary infection. Four patients developed lymphoma. One of them died of hepatic failure and one of them died of severe infection in the process of anti-tumor treatment. Conclusions The clinical feature of CAEBV infection is varied. More attention should be paid to the disease because of its severe complications, poor prognosis and high mortality.