Bai Geng-Hao, Lin Mei-Hwa, Chen Yu-Pei, Chang Tien-Jyun, Chou Sheng-Chieh
Department of Internal Medicine, National Taiwan University Hospital, Taipei City, Taiwan.
Department of Laboratory Medicine, National Taiwan University Hospital, Taipei City, Taiwan.
Thromb J. 2025 May 19;23(1):50. doi: 10.1186/s12959-025-00740-w.
Thrombotic thrombocytopenic purpura (TTP) is an uncommon and life-threatening disorder caused by a deficiency of ADAMTS-13, and eventually leads to microangiopathic hemolytic anemia, severe thrombocytopenia, and organ damages. Acute TTP events could be triggered by infections, or inflammations in the context of ADAMTS-13 deficiency. Recently, several case reports have indicated an association between diabetic ketoacidosis (DKA) and TTP. Here, we present a case with the concomitant presentation of DKA and TTP.
A 37-year-old male with diabetes mellitus presented with typical symptoms of diabetic ketoacidosis. He was managed with an insulin pump and intravenous fluids. However, he developed seizure and progressed to coma, his rapidly deteriorating condition necessitated continuous renal replacement therapy, intubation, and inotropic support. Laboratory data indicated hemolytic anemia and thrombocytopenia, and a blood smear revealed schistocytes. The PLASMIC score was 5, and ADAMTS-13 activity was 2%. The patient was diagnosed with TTP and treated with therapeutic plasma exchange, steroids, and rituximab. His platelet count stabilized above 150,000/µL, and ADAMTS-13 activity progressively improved.
This case report emphasizes the concurrence of DKA and iTTP, presenting the rare complication of acute renal failure in TTP. TTP is a rare and serious disease that requires prompt recognition and management. Concurrent conditions should be considered when calculating prediction scores such as the PLASMIC and French scores.
血栓性血小板减少性紫癜（TTP）是一种由ADAMTS - 13缺乏引起的罕见且危及生命的疾病，最终会导致微血管病性溶血性贫血、严重血小板减少和器官损害。急性TTP事件可能由感染或ADAMTS - 13缺乏情况下的炎症引发。最近，几例病例报告表明糖尿病酮症酸中毒（DKA）与TTP之间存在关联。在此，我们报告一例同时出现DKA和TTP的病例。
一名37岁的糖尿病男性患者出现了糖尿病酮症酸中毒的典型症状。他接受了胰岛素泵和静脉补液治疗。然而，他出现了癫痫发作并进展为昏迷，其病情迅速恶化，需要持续肾脏替代治疗、插管和使用血管活性药物支持。实验室数据显示溶血性贫血和血小板减少，血涂片显示有破碎红细胞。PLASMIC评分为5分，ADAMTS - 13活性为2%。该患者被诊断为TTP，并接受了治疗性血浆置换、类固醇和利妥昔单抗治疗。他的血小板计数稳定在150,000/µL以上，ADAMTS - 13活性逐渐改善。
本病例报告强调了DKA和免疫性TTP的同时出现，呈现了TTP罕见的急性肾衰竭并发症。TTP是一种罕见且严重的疾病，需要及时识别和处理。在计算如PLASMIC和法国评分等预测分数时，应考虑并发情况。
