朱家良 李国华

    [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。

    [关键词] AL型心肌淀粉样变；分期及预后；早期诊断；治疗

    [中图分类號] R542.2 [文献标识码] A [文章编号] 1673-7210(2019)04(c)-0050-05

    Progress in diagnosis and treatment of AL-type myocardial amyloidosis

    Zhu Jialiang1，2 Li Guohua2

    1.Graduate School of Shandong First University， Shandong Provicne， Taian 271000， China； 2.the Second Department of Cardiology， Qianfoshan Hospital， Shandong Province， Ji′nan 250000， China

    [Abstract] 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 ......