6例成年患者的严重爱泼斯坦-巴尔病毒相关噬血细胞综合征

Severe Epstein-Barr virus-associated hemophagocytic syndrome in six adult patients.

作者信息

Elazary Anat Scheiman, Wolf Dana G, Amir Gail, Avni Batia, Rund Deborah, Yehuda Dina Ben, Sviri Sigal

机构信息

Department of Internal Medicine B, Hadassah Medical Center, Jerusalem, Israel.

出版信息

J Clin Virol. 2007 Oct;40(2):156-9. doi: 10.1016/j.jcv.2007.06.014. Epub 2007 Aug 20.

DOI:10.1016/j.jcv.2007.06.014

PMID:17703994

Abstract

BACKGROUND

EBV associated hemophagocytic syndrome (HPS) is an aggressive and potentially life-threatening condition. So far, most EBV associated HPS has been characterized mainly in infants and children in Asian countries.

RESULTS

Here, we report six cases of EBV associated HPS occurring in previously healthy adults in a non-endemic area within a short period of 3 years. All patients presented with fever, hepatosplenomegaly and pancytopenia as well as disturbed liver function tests and coagulopathy. Half were diagnosed as having lymphoma. While EBV-specific serological assays were non-diagnostic in four of the six patients, the presence of EBV DNA in plasma allowed the diagnosis of EBV associated HPS in all patients.

CONCLUSION

EBV associated HPS may be more prevalent in non-Japanese adults than was previously considered. Screening for hemophagocytic syndrome, in adults as well as in children, should include real-time PCR for EBV.

摘要

背景

EB病毒相关噬血细胞综合征（HPS）是一种侵袭性且可能危及生命的疾病。到目前为止，大多数EB病毒相关HPS主要在亚洲国家的婴幼儿和儿童中得到描述。

结果

在此，我们报告了3年内发生在非流行地区既往健康成年人中的6例EB病毒相关HPS病例。所有患者均出现发热、肝脾肿大、全血细胞减少以及肝功能检查异常和凝血功能障碍。半数患者被诊断为淋巴瘤。虽然6例患者中有4例EB病毒特异性血清学检测无法确诊，但血浆中EB病毒DNA的存在使得所有患者均被诊断为EB病毒相关HPS。