Clinical analysis of Epstein-Barr virus-associated hemophagocytic syndrome in children / 中国实验血液学杂志
Journal of Experimental Hematology
;
(6): 460-464, 2013.
Article
in Chinese
| WPRIM
| ID: wpr-332758
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Prognosis
/
Virology
/
Retrospective Studies
/
Risk Factors
/
Herpesvirus 4, Human
/
Epstein-Barr Virus Infections
/
Diagnosis
/
Lymphohistiocytosis, Hemophagocytic
/
Infectious Mononucleosis
Type of study:
Diagnostic study
/
Etiology study
/
Practice guideline
/
Observational study
/
Prognostic study
/
Risk factors
/
Screening study
Limits:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Language:
Chinese
Journal:
Journal of Experimental Hematology
Year:
2013
Type:
Article
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