Badugu Pradeepthi, Idowu Modupe
Division of Hematology, Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, 6411 Fannin Street, Houston 77030, USA.
Case Rep Hematol. 2019 Jan 14;2019:7425320. doi: 10.1155/2019/7425320. eCollection 2019.
Here we report a case of atypical thrombotic thrombocytopenic purpura that presented as an ischemic cerebrovascular accident. A 56-year-old man with multiple cardiovascular risk factors presented with sudden left-sided weakness, slurred speech, and left facial droop. He showed mild improvement when he was treated with thrombolytic therapy according to the hospital stroke protocol. Later in the course, he developed thrombocytopenia followed by schistocytes revealed by peripheral blood smear and other lab abnormalities. Thrombotic thrombocytopenic purpura (TTP) was suspected, and he was treated with total plasma exchange that improved his condition significantly. This case shows that TTP can have unusual and atypical presentations either with the first episode or upon relapse, making diagnosis extremely difficult. Because patients may not present the expected clinical findings, it is important to be aware of variant presentations. In the early stages of the disease, platelet aggregation and thrombus formation may not be widespread, and thrombocytopenia and microangiopathic hemolytic anemia may not be clinically evident. Patients can present soon after the onset of symptoms when the typical laboratory abnormalities may not have had ample time to manifest. Although most other similar cases in the literature had a previous typical presentation of the disease before an atypical presentation, our patient's first presentation was atypical. An atypical presentation of disease in a patient with cardiovascular risk factors may therefore be extremely difficult to diagnose. We believe that TTP should be considered for any patient presenting with stroke and thrombocytopenia.
在此，我们报告一例表现为缺血性脑血管意外的非典型血栓性血小板减少性紫癜病例。一名有多种心血管危险因素的56岁男性，出现突发左侧肢体无力、言语不清和左侧面部下垂。按照医院的卒中治疗方案对其进行溶栓治疗后，他的症状有轻度改善。在病程后期，他出现血小板减少，随后外周血涂片显示有裂红细胞以及其他实验室异常。怀疑为血栓性血小板减少性紫癜，遂对其进行了全血浆置换治疗，病情显著改善。该病例表明，血栓性血小板减少性紫癜无论是首发还是复发，都可能有不寻常和非典型的表现，这使得诊断极为困难。由于患者可能不会出现预期的临床症状，因此了解其变异表现很重要。在疾病早期，血小板聚集和血栓形成可能并不广泛，血小板减少和微血管病性溶血性贫血在临床上可能并不明显。患者可能在症状出现后不久就前来就诊，此时典型的实验室异常可能还没有足够的时间显现出来。虽然文献中大多数其他类似病例在出现非典型表现之前有过该病的典型表现，但我们的患者首次表现即为非典型。因此，对于任何出现卒中及血小板减少的患者，都应考虑血栓性血小板减少性紫癜的可能。
