女性系统性轻链型淀粉样变性伴严重心脏血管事件1例  

A Case of Female Systemic Amyloidosis with Severe Cardiovascular Events

作　　者：黄伟强 丛超 于雪晴 刘秀 Huang Weiqiang;Cong Chao;Yu Xueqing(Department of Cardiology,Yantai Affiliated Hospital,Binzhou Medical University,Yantai,Shandong,264100,China)

机构地区：[1]滨州医学院烟台附属医院心血管内科,山东烟台264100

出　　处：《黑龙江医学》2024年第18期2251-2253,共3页Heilongjiang Medical Journal

摘　　要：系统性轻链型淀粉样变性是由单克隆免疫球蛋白轻链错误折叠形成淀粉样蛋白,沉积于多组织器官,导致组织结构被破坏、器官功能障碍并呈进行性进展的疾病。当系统性淀粉样变性累及心脏时,可能表现为临床异质性,其特征为限制型心肌病、难治性心力衰竭、心律失常,容易导致漏诊和误诊。文章报道1例40岁中年女性,因双下肢水肿进行性加重,进而发生严重的心血管事件合并严重的多系统受累,最终确诊为系统性轻链型心肌淀粉样变性的病例,并以此病例进行深入探讨。Systemic light amyloidosis is a progressive disease,which is caused by the misfolding of light chains of monoclonal immunoglobulin to form amyloid protein and deposited in multiple tissues and organs,leading to the destruction of tissue structure and organ dysfunction.When systemic amyloidosis affects the heart,it may be characterized by clinical heterogeneity,which is characterized by restrictive cardiomyopathy,refractory heart failure and arrhythmia,which may easily lead to missed diagnosis and misdiagnosis.The study reports 1 case of 40 year old middle-aged female who was diagnosed as systemic light chain myocardial amyloidosis due to the progressive aggravation of edema in both lower extremities and the discovery of serious cardiovascular events combined with serious multi system involvement.

关 键 词：心肌淀粉样变性 轻链型 系统性 心力衰竭 

分 类 号：R597.2[医药卫生—内科学]