Mugnai Giacomo, Cicoira Mariantonietta, Rossi Andrea, Vassanelli Corrado
Department of Medicine, Division of Cardiology, University of Verona, Verona, Italy.
Exp Clin Cardiol. 2011 Summer;16(2):51-3.
Primary systemic amyloidosis is a relatively uncommon disease characterized by the production and deposition of pathological insoluble fibrillar proteins in organs and tissues. It has been estimated that between one-third and one-half of all patients with primary amyloidosis experience clinically significant cardiac involvement. The present study reports a case involving a 77-year-old woman with ischemic heart disease who presented to the cardiology department because of syncope due to slow atrial fibrillation. Laboratory tests revealed a monoclonal spike in the gamma fraction and impairment of renal function, normocytic anemia, mild hypercalcemia, hypoalbuminemia and increased levels of beta-2 microglobulin. Suspicion of cardiac involvement was supported by the echocardiographic pattern and increased levels of troponin I and brain natriuretic peptide, along with clinical signs of heart failure and systemic amyloidosis diagnosis, confirmed by abdominal fat aspiration.
原发性系统性淀粉样变性是一种相对罕见的疾病,其特征是在器官和组织中产生并沉积病理性不溶性纤维状蛋白质。据估计,所有原发性淀粉样变性患者中有三分之一至二分之一会出现具有临床意义的心脏受累情况。本研究报告了一例病例,患者为一名77岁患有缺血性心脏病的女性,因缓慢型心房颤动导致晕厥而就诊于心脏病科。实验室检查显示γ球蛋白组分出现单克隆峰,同时伴有肾功能损害、正细胞性贫血、轻度高钙血症、低白蛋白血症以及β2微球蛋白水平升高。超声心动图表现、肌钙蛋白I和脑钠肽水平升高,以及心力衰竭的临床体征均支持心脏受累的怀疑,经腹部脂肪抽吸术确诊为系统性淀粉样变性。