| Objective To explore the clinical characteristics of patients with hemophagocytic lymphocytic histiocytosis(HLH)and retrospectively analyze the treatment and prognosis,so as to improve cognition,early diagnosis,early treatment and prognosis of HLH.Methods This study retrospectively analyzed the clinical data of 62 patients with HLH diagnosed in XXX general hospital from January 2008 to May 2019.All patients were followed up by telephone and analyzed by SPSS 26.0.Results1.Among the 62 patients with HLH,there were 35 males(56%)and 27 females(44%).The ratio of male to female was 1.3:1.2.The causes of sHLH included infection in 23 patients(37.1%),hematological tumors in 13 patients(20.9%),autoimmune diseases in 8 patients(12.9%),and unknown etiology in18 patients(29%).3.The main clinical manifestations of sHLH were fever in 60 patients(96.8%),pulmonary infection in 24 patients(38.7%),gastrointestinal symptoms in 4 patients(3%),jaundice in 1 patient(1.6%),splenomegaly in 49 patients(79%),and hepatosplenomegaly in18 patients(29%).4.Laboratory examination included: hematological system damaged in 58 patients(93.6%),hemophagocytosis in 48 patients(77.4%),the level of AST increased in 48 patients(77.4%),the level of TBIL increased in 40 patients(64.5%),the level of ALB decreased in 53patients(85.5%),the level of TG increased in 26 patients(41.9%),the level of PTA increased in 36 patients(58.1%),the level of FIB decreased in 37 patients(59.7%),the level of CRP increased in 50 patients(80.6%),the level of FER was increased in 58 patients(93.5%).5.The treatment of sHLH: 19 patients(30.6%)were treated with anti infection and other symptomatic treatment,the survival rate was 31.6%;11 patients(17.7%)were treated with glucocorticoid the survival rate was 45.5%;5 patients(8.1%)were treated with glucocorticoid and etoposide,the survival rate was 60%;4 patients(6.5%)were treated with glucocorticoid and cyclosporine,the survival rate was 75%;11 patients(17.7%)were treated with glucocorticoid,the survival rate was 63.6%;3 patients(4.8%)were treated with glucocorticoid,cyclosporine,etoposide and immunoglobulin,the survival rate was 33.3%;4patients(6.5%)were treated with glucocorticoid and immunoglobulin,the survival rate was75%;5 patients(8.1%)gave up treatment,the survival rate was 0.The survival patients were28,the overall survival rate was 45.2%,34 patients died,the overall mortality rate was 54.8%.6.There were significant differences in length of stay,time of diagnosis,duration of fever,hepatosplenomegaly,blood phagocytosis in bone marrow,UREA,CREA,AST,IBIL,APTT,APTT-R,FIB and TT among HLH patients of different ages(P<0.05).7.There were significant differences in length of stay,AST,TBIL,DBIL,IBIL,TP,ALB,CHOL,PTA,TT and FIB among HLH patients with different prognosis(P<0.05).Fibrinogen quantification(P=0.026,OR=2.053)was an independent risk factor of HLH.Conclusion1.The etiology of sHLH is variable,infection is the most common,next is blood system tumor and immune system diseases.2.sHLH is a dangerous disease with high mortality,especially in the minor patients.3.Fibrinogen may be an independent risk factor for the prognosis of HLH patients。... |
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