AL型心肌淀粉样变的诊疗进展  

Progress in diagnosis and treatment of AL-type myocardial amyloidosis

作　　者：朱家良 李国华[2] Zhu Jialiang;Li Guohua(Graduate School of Shandong First University, Shandong Provicne, Taian 271000, China;the Second Department of Cardiology, Qianfoshan Hospital, Shandong Province, Ji′nan 250000, China)

机构地区：[1]山东第一医科大学研究生处,山东泰安271000 [2]山东省千佛山医院心内二科,山东济南250000

出　　处：《中国医药导报》2019年第12期50-53,57,共5页China Medical Herald

摘　　要：心肌淀粉样变性的机制是不可溶性淀粉样蛋白在心肌组织的沉积,进而导致心肌电活动传导受损、心肌肥厚、心肌僵硬度增加、舒张功能下降等心功能紊乱的一种疾病。原发性淀粉样变性和伴发于浆细胞病的淀粉样变所沉积淀粉样物质均是免疫球蛋白轻链,故统称为AL型淀粉样变。心肌淀粉样变患者早期症状不明显,但是随着病情的发展,可出现以心肌舒张功能受限的症状,往往预示着疾病预后不佳。其中AL型心肌淀粉样变较其他类型更为多见,因此本文着重就AL型心肌淀粉样变的早期临床表现、分期及预后和治疗作一综述。The mechanism of myocardial amyloidosis is the deposition of insoluble amyloid in myocardial tissue,a disease that causes cardiac dysfunction such as impaired myocardial electrical activity,cardiac hypertrophy,increased myocardial stiffness,and decreased diastolic function.Primary amyloidosis and amyloidosis associated with plasma cell disease are all immunoglobulin light chains,so they are collectively referred to as AL-type amyloidosis.Early symptoms of myocardial amyloidosis are not obvious,but as the disease progresses,symptoms of myocardial diastolic function may appear,which often indicates poor prognosis.Among them,AL-type myocardial amyloidosis is more common than other types,so this article focuses on the early clinical manifestations,staging and prognosis,treatment of AL-type myocardial amyloidosis.

关 键 词：AL型心肌淀粉样变 分期及预后 早期诊断 治疗 

分 类 号：R542.2[医药卫生—心血管疾病]