Analysis of prognostic risk factors in children with Epstein-Barr virus-associated hemophagocytic syndrome.
OBJECTIVE
This study aims to analyze the prognostic risk factors in children with Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH).


METHODS
Seventy-four EBV-HLH patients were divided into two groups according to the specificity: clinical remission after four-week inductive therapy group and active disease group; death group and survival group. The risk factors that affect early efficacy and prognosis were analyzed.


RESULTS
Overall survival rate of the 74 children was 75.7%, while the recurrence rate was 13.5%. The one-year survival rate was 71.4±5.6%, and the three-year survival rate was 65.9±6.6%, with a median survival rate of 40±19.9 months. The multivariate logistic regression analysis showed that age was the primary risk factor that affected the first 4 weeks alleviation, and the severity of splenomegaly and WBC level upon hospitalization were the risk factors that affected the prognosis. Patients with spleen>4 cm had shorter survival time than those with spleen≤4 cm, and patients with WBC≥3×10(9)/L had longer survival time than those with WBC<3×10(9)/L, which exhibited significant differences.


CONCLUSIONS
Age negatively influences the early remission of EBV-HLH. WBC<3×10(9)/L and spleen>4 cm exhibited high correlation with the prognosis of EBV-HLH.
Department of Pediatrics, Affiliated Sun Yat‑sen Memorial Hospital of Zhongshan University, Guangzhou Guangdong Province, China - hmccdoc@163.com.
H
H
Xue
C
C
Chen
W
W
Li
C
C
Lin
J
J
Fang
Y
Y
Li
H
H
Xu
D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000188:drug therapy / Q000401:mortality / Q000821:virology*; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D007223:Infant; D016015:Logistic Models; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy / Q000401:mortality / Q000821:virology*; D008297:Male; D015999:Multivariate Analysis; D011379:Prognosis; D012008:Recurrence; D012074:Remission Induction; D012307:Risk Factors; D012720:Severity of Illness Index; D013163:Splenomegaly / Q000401:mortality / Q000503:physiopathology / Q000821:virology; D015996:Survival Rate