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An autopsy case of myocardial infarction due to idiopathic thrombotic thrombocytopenic purpura

机译:特发性血栓性血小板减少性紫癜致心肌梗死的尸检病例

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摘要

AbstractThrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder characterized by systemic platelet-von Willebrand factor aggregation, organ ischemia and profound thrombocytopenia. In this report, we describe an autopsy case of a 77-year-old Japanese man diagnosed with idiopathic TTP. He had no history of cardiovascular disease symptoms, such as chest pain, ST segment elevation, and elevation of cardiac enzyme levels, except arrhythmia. The patient suddenly died despite receiving many treatments. On autopsy, macroscopically and microscopically, acute and chronic myocardial infarction manifested as petechiae and fibrotic foci and covered a wide area in the myocardium, including the area near the atrioventricular node. The microthrombi in the small arterioles and capillaries were platelet thrombi, which showed positive results for periodic acid-Schiff stain and factor VIII on immunohistochemical staining. The cause of the sudden death was suspected to be myocardial infarction, including a cardiac conduction system disorder due to multiple platelet microthrombi. Asymptomatic myocardial infarction is an important cause of death in TTP. Therefore, the heart tissue, including the sinus-atrial node and the atrioventricular node, should be microscopically examined more closely in autopsy cases of patients with TTP who experienced sudden death of TTP. This report is a critical teaching case considering that its cause of sudden death may be arrhythmia due to a myocardial infarction including cardiac conduction system disorder by platelet microthrombi.
机译:摘要血栓性血小板减少性紫癜(TTP)是一种威胁生命的疾病,其特征在于全身性血小板-von Willebrand因子聚集,器官缺血和严重的血小板减少。在本报告中,我们描述了一名诊断为特发性TTP的77岁日本男子的尸检病例。除心律不齐外,他没有心血管疾病症状的病史,例如胸痛,ST段抬高和心脏酶水平升高。尽管接受了许多治疗,该患者突然死亡。尸检时,从宏观和微观上看,急性和慢性心肌梗塞表现为瘀点和纤维化病灶,并覆盖了心肌的广泛区域,包括房室结附近的区域。小动脉和毛细血管中的微血栓为血小板血栓,对高碘酸-席夫氏染色和凝血因子VIII的免疫组织化学染色显示阳性结果。猝死的原因被怀疑是心肌梗塞,包括由于多个血小板微血栓引起的心脏传导系统疾病。无症状性心肌梗塞是TTP死亡的重要原因。因此,在经历TTP猝死的TTP患者的尸检病例中,应仔细检查包括窦房结和房室结在内的心脏组织。该报告是一个关键的教学案例,考虑到其突然死亡的原因可能是由于心肌梗塞(包括血小板微血栓形成的心脏传导系统疾病)引起的心律失常。

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