| Background:Thrombotic thrombocytopenic purpura is a kind of disease characterized by microangiopathy,which forms thrombus in microvessels.At the same time,it may be accompanied by fever or nervous system symptoms of different degrees and kidney damage.Ten out of a million people suffer from the disease.About one millionth of the new cases occur.Most of them are antibody-mediated and acquired microvascular diseases.At the time of onset,the precursor symptoms are relatively insidious.TTP is a potentially fatal disease.Patients usually have an acute onset,accompanied by non-specific clinical manifestations.The situation is more dangerous.If not treated in time,the fatality rate can reach 90%.Methods:Among the patients admitted to Peking union medical college hospital from December 2012 to December 2018,74 patients met the admission criteria.The age of the patients was over 14 years old.Patients with definite diagnosis of TTP or no ADAMTS13 positive results but high clinical suspicion of TTP;Patients younger than 14 years of age,incomplete clinical data-or missing laboratory examinations,severe immunodeficiency and malignant tumors were excluded.At the same time,the score of APACHEII was calculated for enrolled patients by medical history review,general data analysis and clinical data in 24 hours of admitted to hospital or ICU.According to the prognosis of the patients,the selected patients were divided into death group and non?death group.After statistical analysis,clinical characteristics and death risk factors of TTP patients were summarized.Results:Among the 74 enrolled patients,51 were first diagnosed as emergency patients and 23 were first diagnosed as non-emergency patients.There were 21 male patients and 53 female patients.Average age 44.28;The average length of stay was 23.72 days.During hospitalization,75.689%of the 56 patients were admitted to ICU,and the average length of stay in ICU was 10.88 days.The death toll of 29 accounted for 39.19 percent.Follow-up results showed that all the patients died during hospitalization or within 7 days after automatic discharge from hospital,and 3 of the discharged patients were considered to have recurrence due to the re-reduction of platelet.There were 1 patient with chronic renal insufficiency and 1 patient with abnormal liver function.The follow-up treatment of discharged patients was regular hormone therapy,and the dose was reduced regularly.Some patients were treated with combined immunosuppressive therapy,and 23patients were treated with combined hormone and immunosuppressive therapy.So far,immunosuppressive therapy has been discontinued.Up to now,12 patients still take hormone therapy.The clinical manifestations,complications and treatment of 74 patients were reviewed.1.Clinical manifestations:①Nervous system:41(55.41%)had headache,52(70.27%)had consciousness disturbance,32(43.24%)had seizures,18(24.32%)had pathological signs,2(2.70%)had meninges irritation,and 21(28.37%)had vomiting.②Blood coagulation:57(77.03%)patients with ecchymosis,and 28(37.849%)patients with other bleeding(such as gastrointestinal bleeding,epistaxis,and gross hematuria).③Other manifestations:fever 52 people(70.27%),skin scleral yellow dye 20 people(27.03%),less urine or edema 21 people(28.38%),cough sputum 11 people(14.86Y),chest pain 14 people(18.92%),abdominal pain diarrhea 12 people(16.22%),Raynaud,s phenomenon 18 people(24.329%).@Rare clinical manifestations:1 person with blurred vision,1 person with chilblain rash.The rash disappeared completely after the patient recovered.2.Complications:74 patients showed respiratory,circulatory and hepatorenal dysfunction complications due to different degrees of acute lesions.Among them,31 were treated with mechanical ventilation due to respiratory failure,30 were treated with vasoactive drugs due to circulatory failure,32 were treated with acute renal insufficiency,9 were treated with blood purification due to renal insufficiency,and 9 were treated with acute liver insufficiency.3.Treatment:all the 74 patients were treated with hormone therapy,57 patients were treated with plasma exchange therapy,and 17 patients were treated with Rituxan.Blood routine,liver and kidney function,coagulation function,heart function,non-specific inflammatory indicators(lactate dehydrogenase,ferritin,procalcitonin,c-reactive protein)and specific tests(blood smear,ADAMST13)were summarized in 74 patients.A total of 72 of the 74 patients were sent for examination,and all or a small number of broken red blood cells were observed,with a detection rate of 97.30%and a positive rate of 100%.ADAMST13 was examined and 47 people were sent for inspection,among which two samples were not traceable,with a submission rate of 63.51%.Among the traceable 45-minute sample results,ADAMTS13 had no activity and 31 cases were positive for inhibitor.ADAMTS activity<10ng/mL9 cases were detected only.ADAMTS13 activity was 100 and inhibitor was negative in 5 cases.According to the results,the analysis results showed that mechanical ventilation,vasoactive drugs,APACHEII score,age,K,TBIL,d-dimer and length of stay were statistically significant between the two groups(P<0.05),compared with other indicators,there was no significant difference(P>0.05).In the multivariate Logistic regression model,the results showed that circulatory failure,APACHEII score>20,d-dimer and length of hospital stay were independent factors affecting the prognosis(P<0.05).Conclusions:1、TTP is an acute,potential and life-threatening thrombotic microvascular disease;2、TTP was mainly manifested as headache,disturbance of consciousness,fever and blood stasis and ecchymosis;3、Clinical and laboratory performance mainly to thrombocytopenia,anemia;The blood smear showed broken red blood cells,and ADAMST13 activity was mainly inhibited,which was mainly manifested by renal involvement in some patients;4、Hormone and plasma exchange are the main treatments at present;5、Circulatory failure,APACHEII score>20 and D-dimer were independent risk factors for TTP prognosis. |
