Thrombotic thrombocytopenic purpura (TTP) typically presents with consumptive thrombocytopenia, non-immune intravascular hemolytic anemia, renal failure, various neurologic findings and fever. It is a clinical syndrome that can be associated with drugs such as ticlopidine, quinine, mitomycin C and cyclosporine, allogeneic stem cell transplantation, pregnancy, infections, autoimmune diseases and metastatic carcinoma. Here, we describe a 48-years- old women presented with full picture of TTP and diagnosed as leiomyosarcoma with further evaluation. She was successfully treated with multiple exchange transfusions and total excision of leiomyoma thereafter. As far as we know, this is the first case of leiomyosarcoma of the uterus presented with TTP. Therefore, the women with TTP, in whom no other cause can be found, should undergo a careful gynecological examination and pelvic ultrasonography should be performed.

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