系统性轻链型淀粉样变性发病机制及诊治进展  

Advances in pathogenesis,diagnosis and treatment of systemic light chain amyloidosis

作　　者：洪伊 张媛媛 李真(综述) 黄湘华(审校) HONG Yi;ZHANG Yuanyuan;LI Zhen;HUANG Xianghua(National Clinical Research Center of Kidney Diseases,Jinling Hospital,Nanjing University School of medicine,Nanjing 210016,China;Southeast University School of Medicine,Nanjing 210029,China)

机构地区：[1]南京大学医学院附属金陵医院(东部战区总医院)、国家肾脏疾病临床医学研究中心全军肾脏病研究所,南京210016 [2]东南大学医学院

出　　处：《肾脏病与透析肾移植杂志》2023年第2期156-162,共7页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家自然科学基金面上项目(82270767)。

摘　　要：系统性轻链(AL)型淀粉样变性是一种罕见的浆细胞疾病,常造成心脏、肾脏等重要器官的功能障碍。近年来,AL型淀粉样变性分子机制研究取得的进展,有助于进一步理解AL型淀粉样变性纤维丝结构特点及心脏和肾脏受损的病理机制。此外,AL型淀粉样变性的诊断及治疗方面方法也有所突破,对改进临床实践,提高诊疗水平具有重要意义。本文将对AL型淀粉样变性的发病机制及诊治进展作一综述。Systemic light chain(AL)amyloidosis is a rare plasma cell disease that often causes dysfunction of important organs such as heart and kidney.In recent years,some progress has been made in the field of molecular mechanism of AL amyloidosis,which helps us further understand structural characteristics of amyloid fibrils in AL amyloidosis and pathological mechanism of heart and kidney damage;In addition,there has been some advances in diagnosis and treatment of AL amyloidosis,which is of great significance for improving clinical practice of AL amyloidosis.This article will review recent progress in pathogenesis,diagnosis and treatment of AL amyloidosis.

关 键 词：系统性轻链型淀粉样变性 发病机制 诊断 分子标志物 免疫治疗 

分 类 号：R597.2[医药卫生—内科学]