Acquired thrombotic thrombocytopenic purpura (
TTP) is caused by
autoantibodies against ADAMTS13.
TTP patients run a rapidly fatal course unless immediate
plasma exchange (PEX) is initiated upon diagnosis. Herein, we report a 72-year-old man with
TTP, which developed after he underwent
artificial blood vessel replacement surgery for an abdominal
aneurysm with impending
rupture. In the
perioperative period, the patient received several
platelet transfusions for severe
thrombocytopenia (minimum platelet count: 0.6×10(4)/μl). Thereafter, he was admitted to our department for rapidly progressing
coma with multiple
cerebral infarctions, and was transferred to the ICU. Based on the tentative diagnosis of
TTP, we immediately began PEX and
steroid pulse
therapy. The diagnosis was confirmed thereafter by markedly reduced ADAMTS13 activity (<0.5%) and his being positive for the ADAMTS13 inhibitor. We performed PEX for five consecutive days and administered high-dose
prednisolone (PSL). On the second hospital day (HD), his platelet count rose along with improvement of his consciousness level. The ADAMTS13 inhibitor was not detected on the 10th HD.
TTP did not relapse and his general condition improved despite tapering of PSL. In this case, by closely monitoring ADAMTS13-related parameters and minimizing the number of
plasma exchanges, the patient was able to achieve a remission without the use of boosting inhibitors.