Objective:To retrospectively summarize and analyze the clinical features of Epstein-Barr virus associatied hemophagocytic lymphohistiocytosis in our hospital in recent 6 years,analyze its clinical features and risk factors affecting early prognosis,and explore effective strategies for early diagnosis and treatment.Methods: Collection of clinical data(general conditions,clinical manifestations,laboratory test results,treatment,outcomes)of children with EBV-HLH newly diagnosed at Shengjing Hospital of China Medical University from January 2012 to December2017.The outcome was divided into the improvement group and the deterioration group for initial treatment for 8 weeks.SPSS22.0 software was used to analyze the statistical data.The measurement data were analyzed by t-test or rank sum test.The count data were analyzed by chi-square test and Logistic multi-factor regression analysis was performed.Result: A total of 70 cases of children,40 males and 30 females,from the city in 27 cases,43 cases in rural areas.The age distribution range is six month to three years old,accounting for 68.6% within 3 years of age.The main symptoms included fever in 70 cases,splenomegaly in 44 cases,liver in 41 cases,superficial lymphadenopathy in 27 cases,rash in 18 cases,active bleeding in 17 cases,and central nervous system involvement in 11 cases.Laboratory examination: 59 cases of WBC reduction,52 cases of ANC reduction,48 cases of HB reduction,65 cases of PLT reduction,59 cases of SF elevation,65 cases of elevated AST,64 cases of ALT elevation,58 cases of FIG reduction,and bone marrow hemorrhage Phenomenon in 60 cases,ANC decreased in 56 cases,TG increased in 21 cases.Treatment: 69 cases of hormones,61 cases of gamma,51 cases of VP-16,8 cases of CSA,58 cases of ganciclovir,6 cases of interferon,42 cases used granulocyte stimulating factor,and 9 cases used plasma exchange.Outcome:43 cases were improved and discharged within 8 weeks of treatment,27 cases wererelieved without hospitalization or died,including 5 cases of gastrointestinal bleeding,4cases of pulmonary hemorrhage,3 cases of intracranial hemorrhage,14 cases of serous effusion,and 5 cases involved central nervous system.The difference between two groups with hemorrhage,serous effusion,jaundice,PLT,AST,TBIL,APTT,FIB,total T cell percentage,application of VP-16 were statistically significant.Multivariate analysis suggested that the significant correlation factors affecting early prognosis in this study were TBIL,FIB,and application of VP-16.After the use of VP-16,the proportion of fever termination and platelet rebound in the improvement group was higher.Conclusion:1.this group of 70 cases of EBV-HLH children slightly more boys,0.5-3 years old ratio is higher.2.EBV-infected persons should be vigilant for EBV-HLH as long-term hyperthermia,enlarged lymph nodes in the liver and spleen,hematopoietic decline with platelet progression,liver function impairment,abnormal coagulation function,and elevated ferritin levels.3.Increased TBIL and decreased FIB may be risk factors that affect early prognosis.Early application of VP-16 can significantly improve the prognosis.No improvement in fever and no platelet recovery after VP-16 may indicate a poor prognosis. |