부산대학교 도서관

Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic hemolytic anemia (MAHA) resulting from severe deficiency of ADAMTS13. TTP is an acute medical emergency which requires early treatment with therapeutic plasma exchange. With the early use of Rituximab along with PLEX, early response is achieved and relapse rate has gone down. There is lack of published data from India regarding treatment and outcome of TTP. We retrospectively analyzed our data of 21 patients of TTP including 4 patients who had TA-TMA. TTP patients were treated with TPE, pulse methylprednisolone and rituximab. After a median follow up of 57.5 months overall survival in TTP patients was 82.3% in our study and one patient relapsed twice but again responded to same treatment. Relapse free survival was 92.8%. The mortality rate in our study in TTP patients was (3/17) 17.6%. The total response rate was 82.3% (14/17). Out of 4 patients of TA-TMA, Only 33.3% patients responded to plasma exchange in TA-TMA while two patients died (2/4) 50%. Immediate TPE and early rituximab are associated with improved survival in TTP patients, however TA-TMA still remains a significant challenge for transplant physicians and more research is needed in guiding the therapy.