|
Background:Thrombotic thrombocytopenic purpura(TTP) is a rare clinical syndrome characterized by thrombocytopenia, microangiopathic hemolytic anemia, with or without fluctuating neurologic or fever or renal abnormalities. TTP is an almost fatal disease with high mortality and relapse rate, the clinical symptoms and signs of which are variable. Hence it is necessary to strengthen the awareness of the pathogenesis, clinical manifestations, diagnosis, and treatment of recurrence or refractory cases.Objective:To investigate the clinical characteristics and therapeutic strategies of TTP and improve the treatment of TTP. Methods:Retrospective analysis of the clinical manifestations, laboratory tests, treatment and prognosis of 47 cases with TTP admitted to our hospital between 1998 and 2010.Results:11 males and 36 females, male:female= 1:3.27, with a median age of 36 years old; 46.8% of acute onset,40.4% of sub-acute onset,12.8% of chronic onset; hereditary TTP 2.1%, primary of TTP 70.2%, secondary TTP 27.7%. For patients with relapsed or refractory, rituximab, vincristine, cyclophosphamide, cyclosporine A, etc. The overall recurrence rate was 17.02%, survival rate was 74.47%, mortality was 25.53%.Conclusion:TTP is more common in female, and most lack of obviously causes. The treatment of plasma exchange combining with corticosteroids is a standard regimen of TTP. The immunosuppressive agents can be used to increase the remission rate of patients with relapsed or refractory TTP, reduce relapse rate and reduce the amount of plasma used. Using PLT count is more accurate to assess the therapeutic response and determine the valid endpoint of plasma exchange cessation. Patients older than 50 years old, severe neurological symptoms such as high fever, coma, and neurological symptoms which continue to increase (P<0.05) on initial TTP presentation are closely related to the death of patients. Coma and neurological symptoms which continue to increase are independent prognostic factors of TTP.
|