Clinical Analysis Of 65 Children With Hemophagocytic Lymphohistiocytosis

Objective:Hemophagocytic lymphohistiocytosis（HLH）is a potentially fatal disease,threatening the lives of patients.If timely treatment is not available,the mortality rate is more than 50%^[1].In this study,we retrospectively analyzed the etiology,clinical manifestations,laboratory indicators and survival of HLH in pediatric inpatients in our hospital to increase the understanding of HLH,so as to confirm the diagnosis of HLH as soon as possible and take timely treatment measures to improve the prognosis.Methods:Collect the general condition（ages,gender）,primary disease,onset symptoms,laboratory indicators,bone marrow morphologyof 65 patients diagnosed with hemophagocytic lymphohistiocytosis who were hospitalized in the Department of Pediatric Hematology of our hospital from January 2015 to December 2019（including those who were transferred to our department for further treatment after diagnosis in other departments）.Analysis was carried out by a retrospective.SPSS 23.0 software was used for data analysis.For measurement data,the normal distribution is expressed as the mean±standard deviation,and the comparison between groups is analyzed by t-test;the measurement data with non-normal distribution is expressed as the median（P25,P75）,and the comparison between groups is performed by non-parametric test.The enumeration data is expressed as a percentage（%）,and the chi-square test is used for comparison between groups.When P<0.05,we think the difference is statistically significant.Results:（1）A total of 65 HLH patients,34 males and 31 females,the male to female ratio was 1.1:1.The age of onset was 3.5±3.1 years old.The number of patients younger than3 years old accounted for 60%.Among 65 cases of HLH,1 case（1.5%）was primary HLH.There were 64 cases（98.5%）of secondary HLH.There were 54 cases（83.1%）of HLH caused by infection,of which epstein-barr virus infection was the most,with 29cases（53.7%）.There were 8 cases（12.3%）of autoimmune disease-related HLH,including 4 cases of Kawasaki disease,3 cases of juvenile idiopathic arthritis,and 1 case of systemic lupus erythematosus.2 cases（3.1%）of tumor-related HLH were T lymphoid non-Hodgkin’s lymphoma and acute lymphocytic leukemia.（2）The clinical manifestations of HLH are diverse,The clinical manifestations of HLH are diverse,100%have body temperature>38.5℃,66.2%liver enlargement,55.4%lymphadenopathy,46.2%respiratory symptoms,44.6%enlargement of spleen,43.1%serous cavity effusion,digestive tract symptoms 38.5%,skin rash 32.3%,neurological symptoms 12.3%,bleeding points 9.2%.（3）The main laboratory indicators are hemocytopenia,AST increased,ALT increased,SF increased,and LDH increased,hemophagocytosis in the bone marrow.Among the diagnostic indicators,SF increased was the most common,accounting for 89.2%,followed by hemophagocytosis in the bone marrow,accounting for 76.9%.General manifestation of hemocytopenia is PLT and ANC reduction.（4）Comparing the age<3 years old group and the age≥3 years old group,WBC,ANC,PLT,TBIL,LDH,SF,and hemophagocytosis in the bone marrow were not statistically significant.The infant group has lower HB,albumin,and FIB than older children,and higher AST,ALT,and TG（P<0.05）.Conclusion:（1）The most common secondary factor of HLH is infection,of which EBV infection is the most common.（2）The clinical manifestations of HLH are diverse.When there is fever,PLT decreased,ferritin increased,it is necessary to suspect whether it is HLH,and take more examination to confirm the diagnosis.（3）The younger the patient was,the lower the HB level and the more severe liver damage are.（4）HLH is a syndrome,not an independent disease.Clinically,it is necessary to pay attention to find primary diseases,like systemic lupus erythematosus and lymphoma.