| Objective:1.To explore the clinical features,treatment and prognostic factors of severe hemophagocytic syndrome(HLH)in children,and to provide references for improving its overall survival rate.2.To analyze the curative effect and prognostic factors of plasma exchange(PE)and/or continuous renal replacement therapy(CRRT)combined with chemoimmunotherapy in children with severe,to provide the basis for the wide application of this method.Methods:1.The clinical data of children diagnosed with severe HLH from January 2014 to April2021 in our hospital were collected,and their clinical features,treatment methods,outcome and prognostic risk factors were analyzed.2.According to whether the blood purification is used or not,the children were divided into blood purification group and non blood purification group,and the difference of efficacy and survival were compared.3.The changes of the complete blood count,liver and kidney function,coagulation function,serum ferritin(SF),Epstein Barr virus(EBV)DNA and cytokines were compared before and after 5-8 days’ treatment in blood purification group.4.To analyze the risk factors influencing the prognosis of blood purification group,and to compare the survival differences between blood purification group and non blood purification group in children with central nervous system(CNS)dysfunction and children with circulatory failure.5.To analyze the correlation between the duration of PE + CRRT and the clinical data at diagnosis in the surviving children of blood purification group.Results:1.All the 83 cases were accompanied with multiple organ dysfunction syndrome(MODS),35 cases(42.17%)had more than 3 organ dysfunction,and 48 cases(57.83%)had2 organ dysfunction.2.In the 83 cases,42 children(50.60%)were treated with blood purification combined with chemoimmunotherapy and 41 children(49.40%)were treated with chemoimmunotherapy.At the end of follow-up,40 cases(48.19%)survived,5 cases(6.02%)lost follow-up,38 cases(45.78%)died.Among the dead children,25 cases(65.79%)were treated with chemoimmunotherapy and 13 cases(34.21%)were treated with blood purification combined with chemoimmunotherapy,31 cases(81.58%)died within 30 days after diagnosis and 27 cases(71.05%)died of MODS.3.CNS dysfunction and not receiving blood purification were independent risk factors for prognosis in children with severe HLH(P < 0.05).4.Before treatment,the indexes of number of organ dysfunction,liver and kidney function(AST,TBIL,CR),myocardial enzyme(CK,CK-MB,LDH)and serum ferritin(SF)in blood purification group were higher than those in non blood purification group(P < 0.05).After treatment,the improvement of AST,LDH,SF,fibrinogen(FIB),the negative rate of EBV and the antipyretic rate in blood purification group were higher than those in non blood purification group(P < 0.05).5.In the blood purification group,electrolyte disorder occurred in 3 cases(7.14%)and blockage occurred in 2 cases(4.76%),the above-mentioned adverse reactions were alleviated by appropriate treatment.6.Compared to before treatment,the indexes of blood routine(HB,PLT),liver and kidney function(ALB,ALT,AST,CR,UA),myocardial enzyme(CK,CK-mb,LDH),coagulation function(PT,APTT,FIB),SF,EBV-DNA,cytokines(IFN-γ,IL-10,IL-6,TNF-α)were significantly improved in blood purification group after treatment(P < 0.05).7.CNS dysfunction and circulatory failure were independent risk factors for prognosis of blood purification group(P < 0.05).but blood purification could improve the survival of children with CNS dysfunction and circulatory failure.8.Among the surviving children in the blood purification group,the duration of PE +CRRT in children with hepatomegaly and EBV infection were longer than those without hepatomegaly and EBV infection,the duration of PE + CRRT in children with circulatory failure at diagnosis was shorter than that in children without circulatory failure.In addition,the levels of IL-6 and IFN-γ were positively correlated with the duration of PE + CRRT,while the levels of PLT,immunoglobulin M(Ig M)and complement C4(C4)were negatively correlated with the duration of PE + CRRT.Conclusion:1.Children with severe HLH are at high risk and have a high mortality rate,and most of them die from MODS at an early stage,CNS dysfunction is an independent risk factor for death,and blood purification is an independent protective factor for improving prognosis.2.Blood purification combined with chemoimmunotherapy can effectively control children’s body temperature in a short time,reduce the levels of cytokines,SF,CRP and EBV-DNA,support important organ function(liver,kidney,coagulation and myocardium)and improve survival rate.,and no serious adverse reactions occurred.It is an effective method for the treatment of severe HLH,and its curative effect is superior to that of chemical immunotherapy alone.3.CNS dysfunction and circulatory failure are associated with poor prognosis in children with blood purification.However,blood purification can reduce the mortality of children with CNS dysfunction and circulatory failure to some extent.4.The duration of PE + CRRT was correlated with liver size,circulation function,EBV infection and the levels of IL-6,IFN-γ,PLT,Ig M and C4 in the surviving children of the blood purification group... |
