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.