A 10-year Retrospective Analysis Of Thrombotic Thrombocytopenic Purpura Center

Objective:Thrombotic thrombocytopenic purpura is a rare critical disease with a mortality of more than 90%.The purpose of this paper is toanalyze and discuss the clinical manifestations,experimental examinations,treatment plans and results of 49 patients diagnosed with TTP in a single center for 10 years,to discuss the clinical characteristics of TTP,to help clinicians make a more accurate diagnosis,to reduce the rate of early misdiagnosis,and toanalyze the risk factors that can be used to determine the prognosis,to compare the therapeutic effect of different treatment regimens,soas to reduce the death rate and improve the prognosis.Methods:SPSS25.0 software was used for dataanalysis.Measurement dataare mainly described in terms of median,mean and range.Those continuous variables subject to normal distribution adopt t test,those not subject to normal distribution adopt rank sum test,and those classified variables adopt chi square test.Stepwise:fordward LR method was used for multivariate logistic regression analysis,The linear trend test Mantel-Haenszel chi-square test was used toanalyze the linear relationship between rank variables,and ROC curve was used to calculate the specificity and sensitivity of relevant indicators.The difference of P<0.05 was statistically significant,and the confidence region represented the 95%confidence interval.Results:1.Among the 49 patients collected,there were a total of 22 patients with rheumatal-immune disease-associated TTP,19 with idiopathic TTP,4 with tumor,3 with hematopoietic stem cell transplantation,and 1 with pregnancy.Therefore,the common TTP types in clinic are mostly rheumatic immune disease-related TTPand idiopathic TTP.Among the 49TTP patients,19 were male patients and 30 were female patients,and the ratioof male to female was about 2:3,which was consistent with the literature reports.The mean age of onset was 49.84 years(16-77 years),and the peak age of TTPbefore 50 years was consistent with literature reports.Of the 49 patients with TTP,9(18.37%)had pentaphylotic,9(18.37%)had tetraphylotic,20(40.82%)had triaphylotic,and 11(22.45%)had diaphylotic.All patients had microangiopathic hemolytic anemiaand thrombocytopenia.It is suggested that the possibility of TTP diagnosis should be highly vigilant in clinical work when the patient encounters the microvascular disease hemolytic anemia with thrombocytopeniaof unknown causes.2.After statistical analysis,it was found that the platelet value less than 23.00×109/Lat the initial onset of the patient indicateda poor prognosis.The analysis of the patients who died in this group found that most of the patients died from complications of the central nervous system.although there was no statistically significant difference in the influence of neuropsychiatric symptoms on the prognosis in the multivariate logistic regression analysis of this group,multiple literatures suggested that neurological symptoms were an independent adverse prognostic factor of TTP.Therefore,it is necessary tobe on high alert for TTP patients with neurological symptoms and initiate plasma exchange therapy immediately.3.Compared with the effective group and the ineffective group,the effective rate of patients undergoing plasmapheresis was 7.64 times that of patients without plasmapheresis.Plasmapheresis can effectively reduce mortality,and is still the most core and important treatment method of TTP until now.once the clinical findings of unknown causes of microvascular disease hemolytic anemia with thrombocytopenia,plasmapheresis should be started immediately.4.In view of the majority of patients for idiopathic or rheumatoid immune disease correlation TTP,glucocorticoid has to inhibit the action of the autoantibodies mediated immune response,low prices,and have the literature effective,this article use glucocorticoid treatment effective rate of 33.33%,suggested in the patients with nocontraindications to the normal use of glucocorticoid treatment.Conclusion:Through the integration and analysis of the clinical dataof TTP inpatients in sichuan provincial people's hospital in the past 10 years,it is found that there is gender difference in this disease,and the prevalence rate of female is higher than that of male.Most of the clinical causes of onset are autoimmune related TTP caused by rheumatic immune disease.Early plasma exchange is the most important therapeutic measure to save the lives of TTP patients.Although early diagnosis is difficult in some patients,as long as there is an unknown cause of microvascular disease hemolytic anemiaand thrombocytopenia,the diagnosis of highly suspicious TTP should be started immediately.Early and active plasma exchange therapy is the most important method to reduce the acute stage mortality,It is suggested that if there is no contraindication of special glucocorticoid use,glucocorticoid treatment can be routinely used in TTP patients to suppress the immune response.The platelet count was less than 23.00×109/L.