Effect Of PE And CRRT Combined With Chemoimmunotherapy On Severe Hemophagocytic Lymphohistiocytosis

Objective:To explore the effect of plasma exchange and continuous renal replacement therapy combined with chemoimmunotherapy in the treatment of severe hemophagocytic lymphohistiocytosis(HLH;also known as hemophagocytic syndrome,HPS).Methods:1)The clinical data of children with severe HLH hospitalized in the pediatric intensive care unit and the pediatric hematology department of our hospital from January 2016 to January 2020 were collected,and their clinical characteristics and prognostic factors were analyzed.2)According to the different treatment methods,the patients were divided into an observation group(PE+CRRT combined with chemical immunotherapy)and a control group(chemical immunotherapy alone),and the curative effect and survival between the two groups were compared.3)The changes of laboratory indexes(cytokines,blood routine,liver and kidney function,myocardial enzymes,TG,CRP,SF)were dynamically observed,and the changes of these indicators(Post)at the end of treatment,2weeks after treatment(2 weeks)and 4 weeks after treatment(4 weeks)in the observation group were compared with those before treatment.4)The patients were divided into fever improved group and fever unimproved group,and multiple organ dysfunction group and few organ dysfunction group according to the number of organ disorders and fever improvement in the observation group respectively.The differences oftreatment,laboratory indexes and survival between different groups were compared.Results:1)In 28 children with severe HLH accompanied with two or more organ dysfunctions,blood system involvement was the most common symptom,followed by respiratory system involvement.PE+CRRT combined with chemoimmunotherapy and fever improvement were independent prognostic factors of severe HLH.2)Comparison between the observation group and the control group: after 7days of treatment,the antipyretic rate of the observation group was 61.9%higher than that of the control group(14.3%),partial thromboplastin time,and levels of ferritin,uric acid,creatinine,activated liver enzyme decreased obviously in the observation group.At the time,levels of fibrinogen and cholinesterase increased significantly in the observation group.And the survival rate of the observation group(66.7%)was higher than that of the control group(14.3%)(P < 0.05).3)The absolute value of neutrophils,and levels of hemoglobin,platelet count,cytokines(SCD25,IFN-?,IL-10,IL-6),ferritin,liver and kidney function index,coagulation function index,myocardial enzyme index and C-reactive protein were significantly improved in the observation group before and after treatment.There was no serious adverse reaction during the treatment of PE+CRRT combined with chemoimmunotherapy.4)The effect of PE+CRRT combined with chemoimmunotherapy in patients with fever improved or not,and in patients with different numbers of organ dysfunction: 1.Whether the number of organ dysfunctions is more than 3 was the best prognostic critical point for the differentiation of survival in patients with severe HLH(P < 0.05),the specificity was 71.43%,and the sensitivity was 85.71%.2.The duration of CRRT was shorter and the survival rate waslower in the multiple organ disorder group when compared to the few organ disorder group.3.Compared to the improved fever group,the duration of CRRT was shorter and the survival rate was lower in the fever unimproved group.4.The duration of CRRT more than 46 h was the best critical point for predicting the improvement of fever(P < 0.05).The specificity was 100% and the sensitivity was 53.85%.Conclusion:PE+CRRT combined with chemoimmunotherapy is an independent prognostic factor in patients with severe HLH,and the survival rate of these patients was significantly improved when compare to patients treated by chemoimmunotherapy alone.By using PE+CRRT combined with chemoimmunotherapy,cytokines,ferritin and EBV-DNA can be quickly removed from the body to support the functions of liver,kidney,lung,heart and blood coagulation,so as to reduce the early mortality of severe HLH and achieve long-term control of this fatal disease.Our results indicated that PE+CRRT combined with chemoimmunotherapy is safe and effective treatment strategy for patients with severe HLH.And the duration of CRRT plays an important role in improving fever and organ dysfunction of patients with severe HLH.