Analysis And Clinical Significance Of The Relationship Between Coagulation Index And Clinical Characteristics In 141 Patients With Hemophagocytic Lymphohistiocytosis

Objective:This study aims to describe the clinical characteristics of patients with hemophagocytic lymphohistiocytosis(HLH)in our hospital,and analyze the relationship between different coagulation indicators,clinical characteristics and prognosis of patients,and then identify the risk factors for the death in patients early,as well as pay attention to HLH patients with coagulation dysfunction.This may play important role in actively controling the symptoms of HLH and improving prognosis of patients.Method:A total of 141 HLH patients who admitted to the First Affiliated Hospital of Nanchang University were included retrospectively.Clinical characteristics were collected and followed up,including general information,laboratory parameters,condition evaluation,bleeding complication and main treatment.We performed a subgroup analysis of the whole patients according to different grouping methods,then use SPSS 22.0 software and Graphpad Prism 7.0 for the statistical analysis and graph-drawing,the P value < 0.05 was considered statistically significant.Results:1.Among 141 HLH patients,the male to female ratio was 1.39:1,and the median age was 47 years(9-89 years).Characteristics of coagulation dysfunction caused by HLH were increase of D-dimer(136 cases)and INR(62 cases)levels,prolongation of prothrombin time(PT)(34 cases),partial thromboplastin time(APTT)(55 cases)and thrombin time(TT)(69 cases),as well as the decrease of prothrombin activity(PTA)(90 cases),fibrinogen(FIB)(64 cases)and platelet(PLT)(111 cases).The incidence of bleeding events in patients was 42.5%,and the common hemorrhagic sites were skin ecchymosis in 28 cases(19.9%),digestive tract bleeding in 25 cases(17.7%),respiratory tract and nosal bleeding in 10 cases(7.09%),respectively.Moreover,4 cases in hemorrhage of gingiva(2.84%),hematuria,ocular and intracranial hemorrhage in 3 cases(2.13%),respectively.2.Overall HLH patients were grouped by using the optimal cut-off value of FIB level(1.68g/L)determined by ROC curve.Patients with FIB≤1.68g/L had shorter median survival time,with higher PT,APTT levels than their counterparts,while the count of PLT was decreased significantly,and more likely to have hemorrhagic events.3.Among 141 patients,ISTH score was 5(0-8)point,76 patients(53.9%)were diagnosed as overt DIC.There were significant differences in the majority of coagulation parameters including PT,PTA,APTT,FIB,D-D,INR,TT and PLT between the overt DIC group and the non-overt DIC group.Furthermore,there were significant differences in the bleeding events and mortality between the two groups.4.The 1-month,3-month,6-month and 1-year overall survival rates of 141 patients were 68.8%,42.3%,35.3%,31.3%,respectively.Comparison of laboratory indicators between the death group and the survival group showed that there were differences in APTT、FIB、PLT levels,ISTH score at initial diagnosis,combined dominant DIC rate and bleeding events,etc.Multivariate analysis showed that patients with age≥29.5 years old(2 points),bleeding events(2 points),APTT≥47.3s(1 point),FIB≤1.68g/L(1 point),and ANC≤1.21×109/L(1 point)were risk factors of poor prognosis.This study formed the prognostic score with these parameters and divided different groups: low risk group(0-2 points),intermediate risk group(3-4points),and high risk group(5-7 points),the 1 year overall survival rate of these three groups was 66.40%,40.00%,and 2.30%,respectively(P<0.0001).Conclusion:Patients with HLH have a high mortality rate.In particular,coagulation dysfunction and bleeding tendency during stages of the disease are common complications of HLH patients who usually had poor prognosis.Decrease in FIB is one of the common abnormal coagulation indicators in HLH patients,which plays a certain role in the prediction of survival outcomes.Once patients with HLH met the diagnostic criteria for overt DIC,the incidence of hemorrahage and the mortality are also improved.In addition,among coagulation indicators,prolonged APTT and hypofibrinogenemia are independent risk factors that affect prognoses of HLH patients.The prognostic score which based on these two indicators combined with ANC,age and bleeding events,can be used to distinguish risk stratification and prognosis of HLH patients.