Successful treatment with cyclosporine in a patient with rituximab-refractory thrombocytopenic purpura
Successful treatment with cyclosporine in a patient with rituximab-refractory thrombocytopenic purpura
复制标题
环孢素成功治疗利妥昔单抗难治性血小板减少性紫癜患者
DOI：
10.11406/rinketsu.62.176
复制
发表时间：
2021
期刊：
影响因子：
--
通讯作者：
俵 功
中科院分区：
文献类型：
--
作者：
名藤 佑真;永春 圭規;岡野 元彦;鈴木 圭;松本 剛史;俵 功
获得性血栓性血小板减少性紫癜（aTTP）是一种以存在抗ADAMTS 13抗体（抑制剂）为特征的危及生命的全身性血栓性微血管病。在这里，我们报告的情况下，难治性aTTP成功治疗环孢素。一位69岁的男性，因血尿及瘀点而转介至本院，他有意识障碍及发热。实验室检查结果显示Coombs阴性溶血性贫血、血小板减少症和肾衰竭。不可检测的ADAMTS 13活性和抗ADAMTS 13抗体（抑制剂）的存在证实了aTTP的诊断。尽管进行了血浆置换并给予泼尼松龙和利妥昔单抗（375 mg/m2），我们仍无法使他的血小板计数恢复到正常水平。因此，他接受环磷酰胺（500 mg/体重）、长春新碱（1.4 mg/m2）、硼替佐米（1.3 mg/m2）和环孢霉素（2.5 mg/kg）治疗。经过环孢霉素治疗后，他的血小板计数逐渐恢复正常。持续环孢素维持治疗导致抑制剂完全消失。难治性aTTP的治疗策略尚未确立。需要进一步研究以建立难治性aTTP的治疗策略。
Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening systemic thrombotic microangiopathy characterized by the presence of anti-ADAMTS13 antibodies (inhibitor). Here we report the case of a patient with refractory aTTP successfully treated with cyclosporine. A 69-year-old man presenting with hematuria and petechiae was referred to our hospital; he was disoriented and febrile. Laboratory results revealed Coombs-negative hemolytic anemia, thrombocytopenia, and renal failure. Undetectable ADAMTS13 activity and presence of anti-ADAMTS13 antibodies (inhibitor) confirmed the diagnosis of aTTP. Despite performing plasma exchange and administering prednisolone and rituximab (375 mg/m 2), we were unable to restore his platelet counts to the normal level. Therefore, he was treated with cyclophosphamide (500 mg/bodyweight), vincristine (1.4 mg/m 2), bortezomib (1.3 mg/m 2), and cyclosporine (2.5 mg/kg). After the cyclosporine therapy, his platelet counts gradually normalized. Continuous cyclosporine maintenance therapy led to complete disappearance of the inhibitor. Therapeutic strategies for refractory aTTP have not yet been established. Further investigations are warranted to establish a therapeutic strategy for refractory aTTP.