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.