title: [Clinical analysis of Epstein-Barr virus-associated hemophagocytic syndrome in children].
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.
authors.affiliation: Department of Pediatrics, Sichuan University, Chengdu, Sichuan Province, China.
authors.forename: Xia
authors.initials: X
authors.lastname: Guo
authors.forename: Qiang
authors.initials: Q
authors.lastname: Li
authors.forename: Chen-Yan
authors.initials: CY
authors.lastname: Zhou
authors.forename: Ya-Ning
authors.initials: YN
authors.lastname: Zhao
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis*; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D007223:Infant; D007244:Infectious Mononucleosis / Q000175:diagnosis / Q000821:virology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000821:virology*; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors