Hong Hyunsoo, Aoyama Yasutaka, Yamamura Ryousuke, Ohta Tadanobu, Mugitani Atsuko, Yamane Takahisa, Hino Masayuki, Matsumoto Masanori, Fujimura Yoshihiro
Department of Hematology, Seichokai Fuchu Hospital.
Rinsho Ketsueki. 2006 Dec;47(12):1528-32.
We report a patient with severe thrombotic thrombocytopenic purpura (TTP) refractory to plasmapheresis who was successfully treated with rituximab. A 57-year-old male patient was referred to our department for further differential diagnosis and treatment of anemia and severe thrombocytopenia. Progressive psychoneurotic symptoms, hemolytic anemia, thrombocytopenia, renal function insufficiency and fever led us to the diagnosis of TTP. ADAMTS13 activity was below 3% and an inhibitor for ADAMTS13 was detected. Treatment with plasmapheresis and high-dose steroid was initiated but without clinical benefit. Two weeks following the initiation of plasmapheresis, we decided to treat the patient with 7 cycles of rituximab. No severe rituximab-related adverse effects were observed. After treatment with rituximab, the disease remitted, and the ADAMTS13 activity level increased. The patient has remained in complete remission for more than 1 year. Our data suggest that rituximab may be the optimal immunosuppressive therapy for refractory thrombotic thrombocytopenic purpura caused by an anti-ADAMTS 13 inhibitor.
我们报告了1例对血浆置换难治的严重血栓性血小板减少性紫癜（TTP）患者，其经利妥昔单抗治疗成功。1例57岁男性患者因贫血和严重血小板减少被转诊至我科进行进一步鉴别诊断和治疗。进行性精神神经症状、溶血性贫血、血小板减少、肾功能不全和发热使我们诊断为TTP。ADAMTS13活性低于3%，并检测到ADAMTS13抑制剂。开始采用血浆置换和大剂量类固醇治疗，但无临床获益。血浆置换开始2周后，我们决定用7个周期的利妥昔单抗治疗该患者。未观察到严重的利妥昔单抗相关不良反应。利妥昔单抗治疗后，疾病缓解，ADAMTS13活性水平升高。该患者已完全缓解超过1年。我们的数据表明，利妥昔单抗可能是由抗ADAMTS 13抑制剂引起的难治性血栓性血小板减少性紫癜的最佳免疫抑制疗法。
