Kawamura Tetsuya, Usui Joichi, Nagai Kei, Sakai Kentaro, Ebihara Itaru, Yamagata Kunihiro
Department of Nephrology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.
Department of Nephrology, St. Mary's Hospital, Kurume, Fukuoka, Japan.
CEN Case Rep. 2018 May;7(1):24-28. doi: 10.1007/s13730-017-0285-y. Epub 2017 Nov 9.
Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening disease. Although plasma exchange (PE) therapy and corticosteroids are standard remission induction and maintenance therapies, some patients are easily refractory and frequently relapse under treatment with this therapy, and require additional treatment. However, there are limited data about additional treatment interventions. We report a case of 56-year-old man who was hospitalized for fever, general fatigue and hemoglobinuria. Owing to the symptoms and the laboratory findings of hemolysis, he was diagnosed with TTP. He was treated with PE therapy and corticosteroids, and the TTP went into remission. However, his TTP relapsed and remission induction was attempted again. As a remission maintenance treatment, we used combination therapy with the purine-synthesis inhibitor mizoribine (MZR) and corticosteroids. The administration of MZR maintained disease activity with no adverse event for long periods and allowed us to gradually reduce the corticosteroids dose. Hence, we propose that MZR is an effective treatment for TTP maintenance.
血栓性血小板减少性紫癜（TTP）是一种罕见但危及生命的疾病。尽管血浆置换（PE）疗法和皮质类固醇是标准的诱导缓解和维持治疗方法，但一些患者在接受这种治疗时很容易出现难治性且频繁复发，需要额外的治疗。然而，关于额外治疗干预的数据有限。我们报告一例56岁男性患者，因发热、全身乏力和血红蛋白尿住院。由于溶血的症状和实验室检查结果，他被诊断为TTP。他接受了PE疗法和皮质类固醇治疗，TTP病情缓解。然而，他的TTP复发，再次尝试诱导缓解。作为缓解维持治疗，我们使用了嘌呤合成抑制剂咪唑立宾（MZR）和皮质类固醇的联合治疗。MZR的给药长期维持了疾病活动且无不良事件发生，并使我们能够逐渐减少皮质类固醇的剂量。因此，我们认为MZR是TTP维持治疗的有效方法。
