系统性免疫球蛋白轻链型淀粉样变性的治疗进展  

Treatment of systemic immunoglobulin light chain amyloidosis

作　　者：付玉娟[1] 周炯[1] 郑敏[1] Fu Yujuan;Zhou Jiong;Zheng Min(Department of Dermatology,The Second Affiliated Hospital of Zhejiang University School of Medicine,Hangzhou 310000,China)

机构地区：[1]浙江大学医学院附属第二医院皮肤科,杭州310000

出　　处：《中华皮肤科杂志》2020年第8期668-672,共5页Chinese Journal of Dermatology

摘　　要：系统性免疫球蛋白轻链型淀粉样变性是因淀粉样蛋白原纤维聚集沉积引起的蛋白质错误折叠疾病,可导致器官不可逆性功能障碍。本文根据疾病危险分层详述本病的系统性治疗方法,低危系统性免疫球蛋白轻链型淀粉样变性患者适用化疗结合自体造血干细胞移植,中高危患者可用蛋白酶抑制剂如硼替佐米、卡非佐米及依沙唑米和免疫调节药物如来那度胺、泊马度胺及新型免疫制剂如达拉图马布、NEOD001等治疗。Systemic immunoglobulin light chain amyloidosis is a protein-misfolding disease caused by the highly organized amyloid fibrillar aggregates that lead to irreversible organ dysfunction.This review summarizes systemic therapies for this disease according to the disease risk stratification.Patients with low-risk systemic immunoglobulin light chain amyloidosis are eligible for chemotherapy combined with autologous hematopoietic stem cell transplantation.Patients with high-risk systemic immunoglobulin light chain amyloidosis can be treated with protease inhibitors(such as bortezomib,carfilzomib and ixazomib),immunomodulatory agents(such as lenalidomide,pomalidomide)and new immunological agents(such as daratumumab and NEOD001).

关 键 词：淀粉样变性 免疫球蛋白轻链 临床方案 

分 类 号：R597.2[医药卫生—内科学]