Exacerbation of cranial nerurological symptoms by platelet transfusion before the diagnosis of thrombotic thrombocytopenic purpura

A 47-year-old woman was transported to our hospital because of vomiting and syncope after breakfast. Physical examination revealed icterus and anemia of bulbar conjunctivas, and abnormal neurological findings were detected. Laboratory data indicated marked anemia and thrombocytopenia (Hb 5.2 g/dl, Plt. 0.6×10(4)/μl), but no leukocyte abnormalities were found. Transaminase was slightly elevated, and serum indirect bilirubin in was also elevated. Based on these data, we initially suspected Evan's syndrome, which involves idiopathic thrombocytopenic purpura with autoimmune hemolytic anemia. So we transfused red blood cells, performed platelet transfusion, and administered steroids, but there was no response to these therapies. On the 4th day of admission, she developed a stroke followed by coma. After the stroke, we diagnosed the case as thrombotic thrombocytopenic purpura (TTP) because laboratory findings showed diminished activity of ADAMTS 13 (a disintegrin-like metalloproteinase with thrombospondin type 1 motifs 13) and ADAMTS 13 antigen. It is important to suspect TTP when hemolytic anemia with thrombocytopenia is observed, and to check the activity and antigen of ADAMTS13 immediately for the diagnosis. Platelet transfusion should be done cautiously in these cases.