系统性轻链型淀粉样变性的诊治进展

Progress of diagnosis and treatment of systemic light chain amyloidosis

淀粉样变性是以淀粉样蛋白异常沉积为特征的一类疾病的统称[1]。系统性轻链(AL)型淀粉样变性为临床最常见类型,常引起心脏、肾脏等机体重要器官功能损害,分别占西方国家及中国所有淀粉样变类型的65%和93%[2]。调查显示,西方国家AL型淀粉样变性发病率为每年每百万9.7~14.0例,我国发病率逐年上升,但尚无大规模流行病学数据[3]。

The amyloidosis are rare diseases that characterized by abnormal deposition of amyloid.Systemic light chain amyloidosis(AL)is the most common type of amyloidosis in clinical practice,which often affects the function of vital organs such as heart and kidney.The incidence of AL has been increased these years in China,but the diagnosis and treatment are relatively insufficient.Imaging methods,biological and genetic indicators are critical to the early diagnosis and the prognosis for AL.Currently,The treatment for light chain amyloidosis is mostly focused on the elimination of malignant monoclonal plasma cells,and some new mechanism drugs targeting amyloid have also shown good efficacy.This article reviews the recent progress of pathogenesis,diagnosis and treatment for light chain amyloidosis.