Clinical study on the early prediction of death for serum markers in infants with hemophagocytic syndrome / 中国小儿急救医学

ABSTRACT

Objective To investigate the association with death for serum parameters at the time of diagnosis and its value in predicting the death in infants with hemophagocytic syndrome (HPS).Methods A retrospective case-control study was conducted on 108 children with HPS who were admitted to our center between July 2005 and July 2012.For each patient,demographic,laboratory data and outcome information were collected.The patients were divided into death and surviving groups based on the follow-up results.The relation between serum markers and death was examined using the COX proportional hazards model and decision tree.Results Of 108 infants with HPS,33 died corresponding to a fatality rate of 30.6％ and 90.3％ of deaths occurred within 8 weeks after diagnosis.Following features were significantly associated with deathwhite blood cells (WBC) ＜5 x 109/L (HR =9.08,95％ CI 3.07 ～ 26.87),hemoglobin ＜80 g/L (HR =6.15,95％ CI 1.68 ～ 22.49),albumin ＜ 28 g/L (HR =4.63,95％ CI 1.12 ～ 7.39),serum ferritin ＞ 1 100 μg/L (HR =3.05,95％ CI 1.28 ～ 16.75),trigeminal ganglion ≥4 mmol/L (HR =2.88,95％ CI 1.51 ～ 8.60),and prothromin time ≥ 16 s (HR =3.60,95 ％ CI 1.28 ～ 7.24),and fever for more than 2 weeks (HR =5.39,95％ CI 1.97 ～ 14.66).Decision tree demonstrated that the probability of death was as high as 100％ for infants with WBC ＜5 x 109/L and hemoglobin ＜ 80 g/L.The odds of dying was still 66.7％ for infants who had WBC≥5 × 109/L but reported trigeminal ganglion ≥4 mmol/L after having fever for more than 2 weeks.Conclusion The first 8 weeks after the onset of HPS is the critical period of treatment.There are several easily available serum predictors of early mortality in HPS infants,particularly the WBC and hemoglobin level,which may help guide treatment decisions.