血栓性血小板减少性紫癜(TTP)是一种少见的综合征,其特征是:微血管病性溶血性贫血、血小板减少性紫癜、神经系统症状、肾脏病变和发热。作者总结了17例 TTP 患者的临床和病理特征、重点研究了心脏,尤其心脏特殊传导组织的病理表现。17例患者中13例有广泛的小血管血栓形成,一些病例心脏有出血和灶性坏死。9例有充血性心力衰竭。血栓形成性微循环的心脏病变和与贫血有关的高心输出量可能造成心脏功能障碍。10例心脏传导系统的一系列组织学切片表明,7例患者有微血栓,其中5例有相关的出血。病变局限于房室结和传导系统的希氏束部,而房室结和束枝病损则少见。2例的心电图提示传导组织内有病损。讨论:心脏内的微血栓是 TTP 的显著特征。根据作者的经验,仅胰腺和肾上腺在此综合征中每例常有血栓形成。尽管在心脏微循环中存在着广泛的血栓,但心肌组织的明显坏死是较小的。作者在17例中观察到8例有多灶性出血和坏死。TTP 时心脏传导组织中 Thrombotic thrombocytopenic purpura (TTP) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenic purpura, neurological symptoms, kidney disease and fever. The authors summarize the clinical and pathological features of 17 TTP patients and focus on the pathological findings of the heart, especially cardiac conduction tissue. Thirteen of 17 patients had extensive small vessel thrombosis and in some cases, hemorrhage and focal necrosis were found in the heart. Nine patients had congestive heart failure. Thrombotic microcirculation of heart disease and anemia associated with high cardiac output may cause heart dysfunction. A series of histological sections of 10 cases of cardiac conduction system showed that there were microthrombi in 7 patients, of which 5 had associated bleeding. Lesions are confined to the bundle branch and conduction system of the His bundle, and atrioventricular node and bundle branch lesions are rare. Two cases of ECG showed conduction tissue lesions. Discussion: Micro-thrombus in the heart is a significant feature of TTP. According to the authors’ experience, only the pancreas and adrenal often have thrombosis in each case in this syndrome. Despite extensive thrombus in the cardiac microcirculation, significant necrosis of myocardial tissue is minor. The authors observed in 8 of 17 patients with multifocal hemorrhage and necrosis. TTP in heart-conducting tissue