儿童EB病毒感染相关性噬血细胞综合征临床特点分析

摘要

本研究分析比较EB病毒相关性噬血细胞综合征(EBV-associated hemophagocytic syndrome,EBV-AHS)与其他病因所致噬血细胞综合征(hemophagocytic syndrome,HPS)的临床特点,了解EBV-AHS患儿预后.回顾性比较我院2000年1月至2007年12月644例EBV-AHS、传染性单核细胞增多症(IM)和其他病因所致HPS患儿的临床症状、体征和实验室检查特点,采用Kaplan-Meier方法分析EBV-AHS的总体生存率.结果表明,①本组IM病例中EBV-AHS发生率为4.2％ (27/644),发病年龄显著低于其他病因所致HPS患儿,以＜3岁的患儿发生率明显高于其他年龄组(P＜0.01)；②＞7岁组患儿肝功能损害明显重于其他两组患儿,LDH、AST和总胆红素水平明显升高,而血浆白蛋白明显低于其他两组；③与其他病因所致HPS患儿相比,男性患儿更为多见,尤其以≤2岁的患儿最为明显,肝功能损害重,尤其以LDH和GGT水平升高为显著；④对EBV-AHS患儿常规抗病毒及对症治疗无效,临床过程凶险,病死率高,本组EBV-AHS患儿病死率高达37.0％,由高到低依次为:＞7岁组、≤3岁组和3-7岁组；⑤HLH-2004方案可显著改善EBV-AHS患儿预后,病死率从50.0％降至18.2％,3年总体生存率显著提高(P=0.032).结论:绝大多数IM患儿呈良性自限性过程,约4.2％患儿进展为EBV-ASH,该病预后凶险,病死率高,HLH-2004治疗方案可显著改善其预后.%The primary infection of Epstein-Barr virus (EBV) may results in hemophagocytic syndrome, known as EBV-associated hemophagocytic syndrome (EBV-AHS), but the clinical risk factors complicating this fatal disease in children with infectious mononucleosis (IM) are unknown. The aim of this study was to identify clinical features of EBV-AHS and to evaluate the curative effect of HLH-2004 protocol. The clinical and laboratory data of 644 IM children including 27 children developed into EBV-AHS and 43 HPS children associated with other diseases were retrospectively analyzed and logistic regression was used to identify the clinical risk factors complicating EBV-AHS. The results showed as follows; (1) the prevalence of EBV-AHS in IM children was 4. 2% (27/644) , and the prevalence in group aged younger than 3 years was higher than in other age groups. The incidence age of EBV-AHS was significantly younger than that of other HPS patients; (2) Liver function damage of group aged older than 7 years was much more severe in HPS patients. (3) Compared with other HPS patients, male patients were more common and liver function damage was severe in EBV-AHS patients, especially in the patients aged at 2 years or yanger. (4) The fatality rate in the EBV-AHS patients was 37.0% (10/27). (5) After treatment with HLH-2004 protocol, the fatality rate in patients with EBV-AHS decreased from 50. 0% to 18.2%, the overall survival (OS) of 3 years significantly increased (P =0.032). It is concluded that IM is a benign self-limited disease, of which only about 4. 2% patients will develop into EBV-AHS. Clinical risk factors identified in this study may be helpful for early diagnosis of IM children with complicated EBV-ASH, the HLH-2004 protocol can obviously improve prognosis of EBV-HPS.