Thrombotic thrombocytopenic purpura with mixed connective tissue disease. A case report

We reported a very rare case of mixed connective tissue disease (MCTD) with thrombotic thrombocytopenic purpura (TTP). The patient was a 24-year-old female who admitted to our hospital in February 2007 because of swelling of her fingers and Raynaud's phenomenon, and was diagnosed as MCTD. Her symptom was improved with the oral administration of prednisolone 10mg/day. In September 2007, her blood examination test showed remarkable thrombocytopenia and hemolytic anemia. A significant number of schistocytes were observed in her peripheral blood smear, and a disintegrin-like and metalloproteinase with thrombospondin typel motifs13 (ADAMTS13) activity in her serum was below the measurement sensitivity, resulted in the diagnosis of TTP. Seven-time plasma exchanges so far cured TTP clinically without any relapse, with remarkable improving of all laboratory data relating to TTP.