Clinical Features And Prognostic Factors Of 40 Cases Of Thrombotic Thrombocytopenic Purpura

Background:Thrombotic thrombocytopenic purpura(TTP)is a kind of rare thrombotic microvascular disease.It is characterized by severe congenital or immune-mediated Willebrand factor metalloproteinase 13(A disintegrin-like and metalloproteinase with thrombo Spondin type 1 motif,member 13,ADAMTS13)deficiency and decreased enzyme activity resulted in unrestricted adhesion and aggregation of Von Willebrand factor(VWF)polymer to platelets and formation of microvascular thrombosis.Leading to microangiopathic hemolytic anemia,thrombocytopenia,and ischemic organ damage from microvascular thromboembolism.TTP diversity of clinical manifestations,the lack of specificity,and complex conditions,and the progress of disaster,if not timely diagnosis and treatment,mortality is extremely high,is still lack of TTP diagnosis on the international gold standard,easy to cause misdiagnosis and missed diagnosis,how to choose the correct early diagnosis and proper treatment and evaluating prognosis of patients with worth further discussing.Objective:By retrospectively analyzing the primary disease,clinical manifestations,laboratory examination results and treatment plan of TTP patients in our hospital,the relevant factors affecting the prognosis of TTP patients were understood.The aim is to improve clinicians’ understanding of this disease,strive for early diagnosis,early intervention and treatment,and improve the prognosis of patients.Methods:Collect the second affiliated hospital of nanchang university in February 2012 to February 2021 hospitalized patients with TTP,all patients diagnosis accord with【2012 expert consensus criteria for the diagnosis of thrombotic thrombocytopenic purpura 】,the general data,clinical manifestations,pathogenesis inducement,laboratory indexes,treatment and prognosis were retrospectively analyzed.Results:(1)In this study,there were 40 TTP patients,including 16 males and 24 females,with a male-to-female sex ratio of 1:1.5.Median age was 53 years(range 18 to 85 years).Age<60 years old: 25 cases,median survival time 50 months;Age ≥60years old: 15 cases,median survival time 35 months,survival analysis between the two groups showed statistically significant difference(P=0.049).(2)Inducing factors: In this study,secondary TTP occurred In 24 cases(60.0%),And the cause was unknown in 16 cases(40.0%).There was no statistical difference between the two groups in survival Analysis(P=0.288).Rheumatic immune diseases were the most common secondary TTP,Accounting for 37.5%.Survival analysis was conducted between the rheumatism immunized group and the nonrheumatism Immunized group,and the results showed that there was no statistical significance between the two groups(P=0.506).(3)Clinical manifestations and laboratory examinations: anemia(100.0%),neuropsychiatric symptoms(92.5%),renal damage(72.5%),fever(60.0%),and bleeding(57.5%)were the main manifestations in 40 patients.The most common laboratory indicators were thrombocytopenia(100.0%),hemoglobin(100.0%),lactate dehydrogenase(100.0%)and reticulocyte ratio(100.0%).There were statistically significant differences in PT,Prothrombin Time,fibrinogen,and Normalized Rate(INR)between the survival group and the death group,with P values of 0.020,0.005,respectively.0.019.Patients in the death group had prolonged PT,elevated fibrin levels,and abnormal INR.(4)In this study,there were 15 cases of ADAMTS13 activity: ADAMTS13 activity <There were 12 cases(80.0%)with 5%,1 case(6.7%)with enzyme activity ranging from 5% to 10%,Twelve patients(66.7%)were detected with ADAMTS13 and 2 cases(13.3%)with enzyme activity ranging from 10% to 20%Inhibitor: 8patients(66.7%)were associated with positive inhibitor.(5)The Plasmic system was used to score,and 80% of the study group were at high risk(6～7points),Which predicted enzyme activity.The positive predictive value of 10% was 86.7%.The patients were divided into two groups for survival analysis based on system score 6,and the difference between the two groups was statistically significant(P<0.001).(6)In this study,24 of the 40 TTP patients survived,with an overall response rate of 60%.During this period,2 patients relapsed,and 1 patient died of disease deterioration.The other one was treated with Plasma exchange(PE)+ glucocorticoid+ rituximab and discharged from hospital.(7)Univariate analysis results: age ≥60 years old(P=0.049),PT >13S(P=0.020),fibrinogen >4g/L(P= 0.005),INR anomaly(P=0.019),PLASMIC<6 points(P<0.001),no PE(P<0.001)was statistically significant and was a risk factor affecting prognosis.In order to exclude the influence of PE on univariate analysis,univariate analysis was also conducted in the PE group.Four times(P= 0.007)and no use of rituximab/immunosuppressant for initial treatment(P=0.004)were the risk factors affecting prognosis.(8)Multivariate analysis showed that INR anomaly(P=0.019),PLASMIC<6points(P<0.001);No PE(P<0.001)was an independent risk factor for prognosis.In the PE group,the results showed that PE<4 times(P=0.017)was an independent risk factor for prognosis.Conclusion:(1)Age ≥60 years is a risk factor affecting prognosis in patients with thrombotic thrombocytopenic purpura.(2)Rheumatoid immune-related diseases are the most common causes of secondary TTP.(3)TTP clinical manifestations are complex and varied,with Hemolytic anemia with microangiopathy,thrombocytopenia,neuropsychiatric symptoms,that is,the typical "triad".The PLASMIC score is helpful for early diagnosis of disease.(4)Adequate plasma exchange can effectively improve the survival rate of patients,and rituximab or immunosuppressant therapy can be chosen for refractory relapsing patients.