A Multicenter Epidemiological Investigation Of Blood Purification Therapy In Children With Hemophagocytic Lymphohistiocytosis

Objective: By a national multi-center questionnaire survey,to provide evidence for the indication of blood purification treatment in children with hemophagocytic lymphohistiocytosis(HLH).Methods: Experts of the Blood Purification Professional Committee of the Chinese Doctor Association Pediatrician Branch drew up the hemophagocytic syndrome registration form,to retrospectively investigate and analysis children with HLH undergoing blood purification treatment in some hospitals in China.Result:(1)Case composition:There were 66 cases of HLH treated by blood purification in 15 tertiary-grade A hospitals,18 of which were from the Department of Nephrology and the rest from PICU;42 males(63.6%),24 females(36.4%),males: females 1.75:1,the median age was 3.23 years old(0.7-13 years old);29 cases in PE group,29 cases in PE+CRRT,5 cases in CRRT,2 cases in PE+HP group,and 1 case in PE+HD group;(2)Etiology: 60 cases(91.0%)of infection,3 cases(4.5%)of lymphoma,3 cases(4.5%)of rheumatic diseases;(3)Clinical manifestations:In PE group,there were 4 cases of respiratory failure(13.8%),and children without heart failure or septic shock.In CRRT group,there were 6 cases of respiratory failure(17.6%),5 cases of heart failure(14.7%),and the previous blood purification treatment of septic rest(8.8%).The cases of organ failure in the CRRT group were more than PE group,and the clinical manifestations of the surviving cases in both groups were alleviated after treatment;(4)Laboratory indicators: blood cells were significantly reduced in the two groups before treatment,and liver function,renal function,coagulation function were significantly impaired.Bilirubin and ferritin in PE group were significantly higher than that in CRRT group,creatinine was significantly lower than that in CRRT group.There were significant differences between the two groups.These were relieved after treatment.The decrease of serum ferritin in PE group was more significant,and was significant difference between the two groups.(5)Outcome: Rresponded:Outcome: Improved: 39 cases(59.1%),died and abandoned treatment :27 patients(40.9%).Among them,PE group improved 18 cases(62.1%),CRRT group improved 19 cases(55.9%).There was no significant difference between the two groups.Conclusion:PE and CRRT have good application prospects in the treatment of hemophagocytic lymphohistiocytosis.For children with high ferritin and severe liver function damage,clinicians often use PE.For children with severe renal impairment,relatively high circulation load and unstable hemodynamics,CRRT and PE were mostly used.Because this investigation is a small sample retrospective study,clinicians still need to adopt an individual program according to the degree of multi-organ dysfunction in children.