Clinical Study Of Early Mortality Prediction By Some Diagnostic Criteria Of Phagocytic Syndrome

Background:Hemophagocytic syndrome(hemophagocyticsyndromes,HPS),also known as hemophagocytic lymphohistiocytosis(hemophagocyticlymphohistiocytosis,HLH),is considered to be a highly unique state of excessive immune activation caused by a variety of pathogenic factors,which was first proposed in 1939.At present,the pathogenesis of the disease is not clear,but in recent years,studies on secondary and sporadic HLH in children and adults have confirmed the role of chronic inflammation,immunosuppression and malignant tumors in the induction and maintenance of HLH.Despite the success of HLH-specific treatment,the fatality rate of adult HLH patients remains high.However,the clinical studies on HLH pay more attention to its diagnosis,while the study on the early prognosis of HLH is rare.Therefore,the development of more accurate tools for early death prediction is of high value for the clinical diagnosis and treatment of HLH.Objective:To explore the clinical value of some serological indexes in the existing diagnostic criteria of hemophagocytic syndrome in predicting the early death of HLH.Methods:A total of 79 patients with HLH admitted to Subei People’s Hospital from January2005 to December 2021 were divided into survival group(n=58)and death group(n=21)according to the prognosis of 30 days after onset.All patients were HLH patients who were diagnosed for the first time and had not yet received treatment.The age,sex,primary disease and main clinical manifestations of all patients were collected.The worst values of hemoglobin(HB),white blood cell(WBC),platelet(PLT),alanine aminotransferase(ALT),aspartate aminotransferase(AST),serum ferritin(SF),triglyceride(TG),fibrinogen(FIB)and C-reactive protein(CRP)were collected and recorded from admission to the diagnosis of HLH.The ultrasonographic results of thoracic and abdominal CT and body surface lymph nodes of all patients were collected.Different statistical methods were used to analyze the differences of indexes among different groups according to the characteristics of the data.COX multivariate analysis was used to screen the independent risk factors associated with early death in HLH.Results:1.The overall median age of HLH patients included in the study was 47.0 years old,with 59.5%of male patients.Infection and hematological tumors were the main causes of HLH patients,of which lymphoma played a major role.Patients in the death group were more likely to have splenomegaly(0.037)and central nervous system symptoms(0.004),but there was no significant difference in other clinical manifestations such as fever,lymph node enlargement and serous effusion between the two groups.2.The counts of HB,WBC and PLT in the death group were significantly lower than those in the survival group(86.46±35.56 vs52.75±22.59)vs 1.75(0.95,2.12),70.5(48.5,91.75)vs 32(6,49),and the level of SF was significantly higher than that in the survival group[4702.5(1618.5,12981)vs 15256(11994,35204),P<0.001].There was no significant difference in AST,ALT,TG,FIB and CRP between the two groups.3.ROC analysis showed that HB,WBC,PLT and SF were all beneficial to the early prognosis of HLH(AUC=0.779,0.708,0.830,0.888).The best cutoff values were Hb<80.06g/L,WBC<3.135×10~9/L,PLT<68.5×10~9/L,SF>10063.5ng/ml,respectively.SF combined with Hb and PLT can further improve the accuracy and positive predictive rate of early death prediction in HLH(AUC=0.938,1/PPV=0.76,1).4.COX multivariate regression analysis showed that Hb,PLT and SF could be used as independent risk factors for early prediction of death in HLH.Conclusion:According to the results of this study,the significance of some diagnostic criteria of HLH-2004 for predicting early death in patients with HLH was analyzed as follows.1.Hemoglobin reduction,leukopenia,thrombocytopenia and elevated serum ferritin can be important factors in predicting death in the early stage of HLH(within 30days).2.Hb<80.06g/L,PLT<68.5×10~9/L and SF>10063.5ng/ml were independent risk factors for death prediction in the early stage of HLH,respectively.3.The combination of three factors,Hb<80.06g/L,PLT<68.5×10~9/L and SF>10063.5ng/ml,is more significant for the prediction of early death in HLH,and the positive prediction rate is extremely high,reaching 100%,suggesting that the patient’s condition is more critical and the prognosis is extremely poor,and the probability of death within 30 days is extremely high,and more targeted and effective treatment should be carried out early.