Clinical Analysis Of 164 Cases Of Hemophagocytic Lymphohistiocytosis

Objective: To retrospectively analyze the clinical characteristics of different disease-related HLH,evaluate the role of H-score in the diagnosis of HLH,assess the efficacy of different treatment,and explore the factors that could affect the outcome.Method: The complete clinical data of 164 HLH patients and 66 patients who were initially suspected of HLH but were diagnosed with systemic inflammatory response syndrome(SIRS)from January 2007 to December 2016 in Tongji Hospital of Wuhan were collected.1)HLH patients were divided into infection-related HLH(?-1),EBV-related HLH(?-2),malignancy-related HLH(?-3),autoimmune-related HLH(?-4)according to the cause of HLH,the main clinical features of the four groups were compared.2)The initial H-score of 71 adult HLH patients(?-1)and 66 non-HLH patients(?-2)were compared.3)142 rHLH patients were divided into immunosuppressive therapy group(?-1)and chemotherapy group(?-2)according to the treatment,the efficacy of two group were compared.4)Multivariate logistic regression analysis was used to analyze the clinical data of 142 rHLH patients to explore the factors that could affect the outcome of HLH.SPSS22.0 software was used for statistical analysis.Results:1.The rate of hepatomegaly was higher in ?-1 and ?-2(p<0.001).In ?-4,the rate of splenomegaly was lower(p=0.033),and the rate of rash was higher than other three groups(p<0.001).The levels of WBC,NEUT and PLT were higher in-1 and-4.The level of TG was higher in-2.The levels of GLO and ESR were higher in-4,and LDH were higher in-3 than that in other groups(p <0.001).2.H-score in ?-1 was significantly higher than that in ?-2(210.23±47.02 VS.105.8±27.53).The sensitivity was 94.37%,and the specificity was 93.94% when 138 was as the cut-off value of H-score.3.In ?-2,the treatment efficiency was 80.5% after 2 weeks' treatment(55.4% in ?-1),and the CR was 53.2% after 4 week' treatment(13.8% in ?-1).The early death rate of group ?-2 was 7.8%(47.7% in ?-1)(p<0.01).4.It has been shown in multivariate regression analysis that the time of being hospitalized to being treated(OR=1.304),AST(OR=1.005),albumin(OR=1.409)and decreased level of ferritin(OR = 0.005)may be factors that could affect the outcome.Conclusion:1.Each rHLH caused by different reasons had special characteristic,which may help us to early identify the primary cause in clinical work.2.H-score may help to assess the risk of HLH,the sensitivity and specificity of was high if diagnosis threshold was 138.3.In the treatment of HLH,chemotherapy could improve the CR rate,treatment efficiency and early survival rate.4.The time of diagnosis and treatment,increased AST,hypoalbuminemia,the decreased level of ferritin may be the factors that could affect the outcome of HLH.