轻链型淀粉样变性的诊断及治疗进展  

Progress of diagnosis and treatment of light chain amyloidosis

作　　者：林泽宇 陈文明[1] Lin Zeyu;Chen Wenming(Department of Hematology,Beijing Chao-Yang Hospital,Capital Medical University,Beijing 100020,China)

机构地区：[1]首都医科大学附属北京朝阳医院血液科,100020

出　　处：《白血病．淋巴瘤》2019年第1期57-60,共4页Journal of Leukemia & Lymphoma

基　　金：国家自然科学基金(81500164).

摘　　要：系统性淀粉样变性是由免疫球蛋白重链或轻链错误折叠并于胞外沉积为淀粉样纤维导致的,其最常见的形式是轻链型淀粉样变性,可造成重要器官的功能损害。特定生物标志物及淀粉样物质成像可于疾病早期提示器官损伤,从而阻止终末期器官功能衰竭。联合疗法是轻链型淀粉样变性治疗的发展方向,针对克隆性轻链与淀粉样沉积的靶向疗法,及可调控轻链聚集与水解的新药研究正在发展。文章就轻链型淀粉样变性的诊断及治疗进展进行综述。Systemic amyloidosis is caused by misfolding of heavy or light chain in immune globulin and extracellular deposition of proteins as amyloid fibrils.The most common form is light chain amyloidosis,which results in dysfunction of vital organs.Specific biomarkers and amyloid imaging can prompt organ dysfunction at early diagnosis and prevent the organ failure at end stage.Combination therapy is the direction of light chain amyloidosis therapy in the future.The studies on the target therapy concerning clone light chain and amyloid deposition,and new drugs that can control light chain gathering and hydrolysis are under exploration.This paper reviews the treatment progress of light chain amyloidosis.

关 键 词：淀粉样变性 诊断 治疗 

分 类 号：R597.2[医药卫生—内科学]