Clinical Analysis Of 39 Patients With Acquired Thrombotic Thrombocytopenic Purpura

Purpose:Thrombotic thrombocytopenic purpura(TTP)is a rare clinical disease and one of the clinical critical illnesses.TTP usually progresses rapidly and has a high mortality rate of more than 90% if not treated effectively.In order to understand the current diagnosis and treatment status of TTP in our center,and to provide meaningful suggestions for the future diagnosis and treatment,as well as to improve the diagnosis and treatment level of TTP and reduce the mortality and recurrence rate of the disease,we retrospectively analyzed the clinical features,treatment plan,test results and outcomes of 39 patients with acquired thrombotic thrombocytopenic purpura in our center to find the differences between the survival group and the death group,the recurrence group and the nonrecurrence group.Method:We retrospectively analyzed the clinical data of 39 patients diagnosed with TTP in Hematology Department of Hunan Provincial People's Hospital from January 2014 to December 2018.The follow-up data including clinical manifestations,general examinations,laboratoryfindings,treatment options,prognosis and recurrence were included.Chisquare test was used to compare the clinical indexes of patients in the death group,the survival group,the recurrence group and the nonrecurrence group so as to find out the factors affecting the prognosis and recurrence of the patients.The odds ratio(OR)and 95% Confidence Interval(CI)were calculated by using logistic regression.Statistics were statistically significant at P < 0.05 using SPSS 20.0 and Stata SE 14.0statistical software.Result:1.Among the 39 patients with TTP,all of them had acquired TTP,including 15 males(38.5%)and 24 females(61.5%).The male to female ratio was 1:1.6,and the age ranged from 34 to 84 years old.The average age was 60.5 years and the median age was 64 years.Only 28.2% of patients had typical "five-signal" symptoms of TTP,including fever,thrombocytopenia,microvascular pathological hemolytic anemia,impaired renal function,and neurological symptoms.However,among all single-group symptoms,thrombocytopenia(100%),microvascular pathological hemolytic anemia(87.2%),and neuro-psychotic symptoms(84.6%)were the three most common symptoms.2.Analyzing the results of the laboratory examinations in all patients,we found that all patients had a decrease in platelet count and an increase in lactate dehydrogenase and broken red blood cell count.87.2% ofpatients had a decrease in hemoglobin,and 51.3% had a rise in serum creatinine.In addition,36 of 39 patients were tested for ADAMTS13 activity.All the patients were tested for a decrease in ADAMTS13 activity and 35 of them had ADAMTS13 activity values less than10%.3.Of the 39 patients,15 died,2 of whom died within 24 hours of admission without any treatment,a total of 24 patients were discharged after treatment,A total of 6 patients(24% of the survival group)had recurrence by December 31,2018,and the recurrence time ranged from 1to 13 months.4.By comparing the survival group and the death group,we found significant differences in serum creatinine value,lactate dehydrogenase,and Body mass index(BMI)between the two groups.Patients in the death group had significantly higher Serum creatinine,lactate dehydrogenase,and BMI than those in the survival group,suggesting that patients at admissionfacing higher risk of death with higher serum creatinine,higher lactate dehydrogenase,and higher BMI.Conclusion:1.Of the 39 patients,15 died,with an overall mortality rate of approximately 38.4%.37 patients underwent plasmapheresis,and 13 of them died.A total of 6 patients were treated with rituximab and nopatients died,which suggests a significant reduction in mortality among patients treated with Rituximab needs more case to comfirm.2.Comparing the clinical data of the survival group and the death group,we found that the age of the patients in the death group and the serum creatinine value,lactate dehydrogenase and BMI were significantly higher than those in the survival group.Therefore,such patients should be given more attention.