原发型心肌淀粉样变的治疗进展  

An update for treatment of primary cardiac amyloidosis

作　　者：程仙 张刘燕 许菲 徐东杰 

出　　处：《临床心血管病杂志》2017年第5期407-410,共4页Journal of Clinical Cardiology

基　　金：江苏高校优势学科建设工程一期项目[No:XK05200903(NG09)]

摘　　要：[提要]原发型即免疫球蛋白轻链型(AL型)淀粉样变是最常见的心肌淀粉样变类型,早期诊断技术及治疗方案的进展,极大改善了原发型心肌淀粉样变患者的预后。自体外周造血干细胞移植可显著延长患者的生存期限,但干细胞移植仅适用于一部分患者。目前马法兰联合地塞米松方案因其良好的耐受性,已成为不适合移植患者的一线方案。近来,蛋白酶体抑制剂及免疫调节剂为复发难治或无法移植的原发型心肌淀粉样变患者带来了新的希望。Primary cardiac amyloidosis is the most common type of cardiac amyloidosis. The development of early diagnosis and therapy greatly improve the prognosis of primary cardiac amyloidosis. Autologous hematopoietic stem cell transplantation can obviously prolong the survival time of patients, but is suitable for a part of patients. Because of its well tolerance, the combination of melphalan and dexamethasone has been the first-line treatment for patients who are not suitable for stem cell transplantation. In addition, proteasome inhibitors and immune regulator play an important role in treatment of refractory primary cardiac amyloidosis.

关 键 词：心肌淀粉样变 干细胞移植 马法兰 地塞米松 

分 类 号：R542.2[医药卫生—心血管疾病]