2012 Volume 3 Issue 1 Pages 78-81
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening generalized disorder. Most of the patients with TTP achieve remission following plasma exchange (PE). This report presents the case of a patient with refractory TTP that was successfully treated with PE and rituximab. A 47-year-old female was referred to our hospital due to general fatigue and slurred speech. She had a slight fever at the time of admission (37.2℃) without any change in consciousness. Her blood examination showed a hemoglobin concentration of 7.0g/dL, platelet counts of 0.7×104/μL, LDH level of 1,555 IU/μL, serum creatinine level of 0.78mg/dL, plasma ADAMTS13 activity of＜0.5％, and anti-ADAMTS13 inhibitor level of 4.8 Bethesda unit/ml. Her platelet counts and ADAMTS13 activity were increased by PE on day 3. However, her platelet counts and ADAMTS13 activity decreased again with an elevation of anti-ADAMTS13 inhibitor on day 7. Therefore, she was treated with PE and rituximab. She achieved clinical remission following the administration three courses of rituximab. These results indicated that daily measurement of ADAMTS13 activity and its inhibitor level were useful to identify patients that require administration of rituximab to reduce the times of PE and improve the prognosis of patients with TTP.
