The Clinical Studies Of TTP Patients With Neurological Manifestations As Prominent Symptoms

Objective:To comprehensively analyze the clinical characteristics,treatment strategies and outcome of thrombotic thrombocytopenic purpura(TTP)presenting neurological manifestations as prominent symptoms.Methods:We performed a retrospective survey of all the 63 patients with TTP from June 2010 to June 2018 in our database.Describe the clinical characteristics of 25 patients with TTP presenting neurological manifestations as prominent symptoms.Results:In our study,the median age was 55(range,22-84),and the ratio of male to female was 1:1.5.18 patients(72.0%)presenting neurological symptoms as initial manifestations,and 5 patients(27.8%)were misdiagnosed as stroke events.Only 6 cases(24.0%)had typical pentalogy of TTP,including thrombocytopenia(100%),microangiopathic hemolytic anemia(76.0%),renal abnormalities(64.0%)and fever(36.0%).Plasma ADAMTS13 activity was detected in 18 patients(72.0%)with ADAMTS13 deficiency confirmed.16 patients(64.0%)completed cranial imaging examination,and the detection rate of MRI(80.0%)was higher than CT(33.3%).Intracranial lesions were found in 10 cases(62.5%),including cerebral infarction(70.0%),cerebral hemorrhage(20.0%)and brain metastasis(10.0%),and 50%of them completely disappeared after treatment.24 patients(96.0%)accepted plasma exchange with response of 54.2%(13/24).12 patients(48.0%)died in our study,and the level of troponin I,total bilirubin and indirect bilirubin showed significant differences(P=0.027,P=0.019,P=0.007).At a median follow-up time of 51 months,1 patient relapsed and 1 patient had slight hemiplegia after cerebral infarction.Conclusion:The neurological manifestations of TTP patients are atypical and always be misdiagnosed.The determination of plasma ADAMTS13 activity is helpful for early diagnosis.The intracranial lesions are reversible,and MRI has higher diagnostic value than CT.Plasma exchange is the preferred treatment of TTP,and the therapeutic alliance with immunosuppressive agents and Rituximab may significantly improve the outcome of relapsed and refractory patients.The high level of troponin I,total bilirubin and indirect bilirubin may be associated with poor prognosis.