Analysis Of Clinical Characteristics And Prognostic Factors Of 44 Cases Of Thrombotic Thrombocytopenic Purpura

Objective: Thrombotic thrombocytopenic purpura(TTP)is a rare life-threatening thrombotic microangiopathy,characterized by microangiopathic hemolytic anemia,severe thrombocytopenia,with or without end organ damage.The application of plasma exchange has greatly reduced the mortality rate of TTP patients,but some patients still have poor efficacy in plasma exchange and there is a risk of recurrence.Comprehensive analysis of the clinical manifestations,laboratory tests,treatment strategies and outcomes of 44 patients with thrombotic thrombocytopenic purpura diagnosed in our hospital from April 2010 to April 2020,aiming to understand the diagnosis and treatment status of our hospital TTP patients Risk factors that affect the prognosis of patients,improve the understanding of risk factors in clinical work,strengthen detection,and adjust treatment strategies in time to further improve the prognosis of TTP patients.Methods: The clinical data of 44 patients with thrombotic thrombocytopenic purpura diagnosed in the First Affiliated Hospital of Nanchang University from April 2010 to April 2020 were retrospectively analyzed.Descriptively analyze the clinical characteristics of 44 TTP patients,analyze the clinical indicators of survival group(n = 21)and death group(n = 23),and find out the risk factors that affect the prognosis of TTP patients.Results: 1.General clinical features: Among the 44 patients with TTP,there were 17 males and 27 females,with a male-to-female ratio of 1.6.The median age of onset was 47.5 years(12-81).;all 44 cases were acquired TTP,its secondary TTP accounts for 43.7%,the main causes are rheumatic immune diseases,infections,malignant tumors,pregnancy,induced after surgery,etc;idiopathic TTP accounts for 56.3%.At admission,the typical "five syndromes" accounted for only 18.2% of the clinical manifestations,and the typical "triple syndromes" accounted for 59.1%.Among them,the single group of symptoms was thrombocytopenia(100%)and microvascular hemolytic anemia(86.4%)And neuropsychiatric symptoms(86.4%)are the most common,some patients have fever(60.4%),and a small number of patients have renal impairment(29.2%).In the laboratory examination,all patients showed thrombocytopenia,increased lactate dehydrogenase,and broken red blood cells(> 1%)found in peripheral blood smears;Only 5 of 44 TTP patients were tested for ADAMTS13 activity at the first visit,2 cases < 5%,3 cases were sent for examination after plasma exchange,and 7 patients were tested for ADAMTS13 activity when they were admitted to hospital after plasma exchange,all of them were less than 10%.Seventy-five percent of TTP patients had a PLASMIC score of a high risk of ADAMTS13 deficiency.2.Treatment and outcome: of the 44 patients with TTP,21 were effective,with an overall effective rate of 47.7%.The overall effective rate of patients treated with plasma exchange was 54.5%,and that of patients treated with immunosuppressant or CD20 monoclonal antibody was 80%.The overall effective rate of patients without plasma exchange was only 27.3%,and the effective rate of patients treated with plasma transfusion was higher than that of patients without plasma transfusion(40% vs 16.7%).Of the 21 patients who survived,7 had relapse again,with an overall recurrence rate of 33.3%,a median recurrence time of 11.2 months,and a range of 1.2 to 31.7 months.3.Analysis of prognostic factors: univariate analysis of various indicators of survival group and death group,of which there is no potential cause(P = 0.017),electrocardiogram has ischemic changes(P = 0.024),and typical clinical "five syndromes"(p = 0.013),admission diagnosis(p = 0.036),lactate dehydrogenase did not decrease by 50% in 5 days(P <0.001),prolonged APTT(P = 0.012)and elevated CK-MB(P=0.037)are risk factors that affect the efficacy of TTP patients;proceed COX regression analysis showed that the ECG had ischemic changes on admission(HR = 2.16,P = 0.046),lactate dehydrogenase did not decrease by 50%(HR = 0.25,P <0.0001)and the typical "five syndromes"(HR = 3.516,P = 0.035)are independent risk factors that affect the efficacy of TTP patients.Conclusion: 1.Thrombotic thrombocytopenic purpura is a medical emergency,with less onset in clinical typical "five syndromes".When unexplained thrombocytopenia,hemolytic anemia,or neuropsychiatric symptoms occur,TTP is considered and dynamic detection of peripheral blood broken red blood cells At the same time,combined with the PLASMIC score is helpful for early diagnosis and timely initiation of TPE treatment.2.Plasma exchange and hormones are still the cornerstones of TTP treatment.When TPE treatment is not possible,plasma transfusion can be given.For refractory patients,immunosuppressive agents or CD20 monoclonal antibodies should be added in time to improve the remission rate.3.Analyze the patients in the survival group and the death group and find that the typical clinical "five syndromes",electrocardiogram with ischemic changes and active treatment for 5 days without LDH reduction of 50% are independent risk factors that affect the efficacy of TTP.When the above risks occur Factors suggest that there is a greater risk of death,and treatment strategies should be adjusted in time.4.TTP shows a tendency to relapse,After remission,ADAMTS13 activity and inhibitor titer should be monitored.When ADAMTS13 activity is found to be significantly reduced,it should be actively treated to prevent relapse.