Takamatsu Ko, Wannaphut Chalothorn, Benavente Kevin, Nishimura Yoshito, Chong Christina
Department of Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, USA.
Division of Hematology and Oncology, Mayo Clinic, Rochester, Minnesota, USA.
Eur J Case Rep Intern Med. 2025 Jul 29;12(9):005628. doi: 10.12890/2025_005628. eCollection 2025.
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening hematologic emergency caused by ADAMTS13 deficiency, leading to microvascular thrombosis, haemolytic anaemia, thrombocytopenia, and end-organ damage. Neurological symptoms occur in up to 90% of cases and are frequently misdiagnosed as stroke. Prompt recognition and treatment reduce the mortality rate from over 90% to 10-20%.
A 70-year-old woman with no significant medical history presented with acute aphasia and right-sided facial and upper extremity numbness. Five days prior, she had experienced fatigue, headache, and bruising. On admission, she had stable vitals with mild aphasia and purpuric ecchymoses noted on examination. Laboratory findings revealed haemolytic anaemia (haemoglobin 7.8 g/dl, lactate dehydrogenase 875 U/l, total bilirubin 2.4 mg/dl), thrombocytopenia (35 ×10/l), and unremarkable coagulation studies (partial thromboplastin time 30 sec, prothrombin time-international normalized ratio 1, fibrinogen 370 mg/dl, D-dimer 2.15 ng/ml). Peripheral smear showed no schistocytes but noted 2+spherocytes. Stroke was ruled out with brain magnetic resonance imaging/magnetic resonance angiography. Given the bicytopenia with possible haemolysis along with new neurological symptoms, TTP was considered in the differential diagnosis. A PLASMIC score of 5 suggested a high probability of TTP. Plasma exchange and corticosteroids were initiated within 5 hours of presentation. ADAMTS13 activity <1% confirmed the diagnosis 4 days after presentation. After five plasma exchange sessions, the patient's platelet count normalized and neurologic deficits resolved by discharge.
This case underscores the importance of considering TTP in stroke mimics, even in the absence of schistocytes, if clinical suspicion is high. Early recognition and initiation of treatment significantly improve patient outcomes.
Start plasmapheresis and high-dose steroids immediately for suspected thrombotic thrombocytopenic purpura (TTP), even if schistocytes are absent when clinical suspicions are high.Recognizing TTP's neurological symptoms mimicking stroke is crucial to avoid misdiagnosis and ensure timely treatment.
血栓性血小板减少性紫癜（TTP）是一种由ADAMTS13缺乏引起的危及生命的血液学急症，可导致微血管血栓形成、溶血性贫血、血小板减少和终末器官损伤。高达90%的病例会出现神经症状，且常被误诊为中风。及时识别和治疗可将死亡率从90%以上降至10%-20%。
一名70岁女性，无重大病史，出现急性失语及右侧面部和上肢麻木。5天前，她曾感到疲劳、头痛和出现瘀伤。入院时，她生命体征稳定，有轻度失语，检查时发现有紫癜瘀斑。实验室检查结果显示溶血性贫血（血红蛋白7.8 g/dl，乳酸脱氢酶875 U/l，总胆红素2.4 mg/dl）、血小板减少（35×10⁹/l），凝血检查无异常（活化部分凝血活酶时间30秒，凝血酶原时间-国际标准化比值1，纤维蛋白原370 mg/dl，D-二聚体2.15 ng/ml）。外周血涂片未见破碎红细胞，但可见2+球形红细胞。脑磁共振成像/磁共振血管造影排除了中风。鉴于存在可能溶血的双血细胞减少以及新出现的神经症状，鉴别诊断中考虑了TTP。PLASMIC评分为5表明TTP可能性高。在就诊后5小时内开始进行血浆置换和使用皮质类固醇。就诊4天后，ADAMTS13活性<1%确诊了该病。经过5次血浆置换治疗后，患者血小板计数恢复正常，出院时神经功能缺损症状消失。
该病例强调了即使在没有破碎红细胞的情况下，若临床怀疑度高，在疑似中风的病例中考虑TTP的重要性。早期识别和开始治疗可显著改善患者预后。
对于疑似血栓性血小板减少性紫癜（TTP），即使临床怀疑度高但外周血涂片未见破碎红细胞，也要立即开始血浆置换和大剂量使用类固醇。认识到TTP类似中风的神经症状对于避免误诊和确保及时治疗至关重要。
