Clinical analysis of Epstein-Barr virus-associated hemophagocytic syndrome in children.
10.7534/j.issn.1009-2137.2013.02.042
- Author:
Xia GUO
1
;
Qiang LI
;
Chen-Yan ZHOU
;
Ya-Ning ZHAO
Author Information
1. Department of Pediatrics, Sichuan University, Chengdu, Sichuan Province, China.
- Publication Type:Journal Article
- MeSH:
Adolescent;
Child;
Child, Preschool;
Epstein-Barr Virus Infections;
diagnosis;
Female;
Herpesvirus 4, Human;
Humans;
Infant;
Infectious Mononucleosis;
diagnosis;
virology;
Lymphohistiocytosis, Hemophagocytic;
diagnosis;
virology;
Male;
Prognosis;
Retrospective Studies;
Risk Factors
- From:
Journal of Experimental Hematology
2013;21(2):460-464
- CountryChina
- Language:Chinese
-
Abstract:
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 younger. (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.
