免疫调节剂治疗系统性轻链型淀粉样变性

Current status of immunomodulatory drugs in treatment of sytematic light chain amyloidosis

系统性轻链型淀粉样变性(AL淀粉样变性)是最常见的系统性淀粉样变性。目前AL淀粉样变性的治疗尚无统一的标准,方案选择主要取决于患者病情严重程度。研究显示,免疫调节剂(IMiDs)通过多种机制发挥抗肿瘤作用,在初治及复发难治性AL淀粉样变性的治疗中均有一定的疗效。本文将对临床常用的沙利度胺、来那度胺及泊马度胺等三种IMiDs在AL淀粉样变性治疗中的现状与进展进行综述,为临床治疗提供参考。

Systematic light chain amyloidosis （ AL amyloidosis） is the most common of the systemic amyloidosis. There is no treatment standard yet.The choice of treatment mainly depends on the severity of the disease.lmmunomodulatory drugs （IMiDs） exert anti-tumor effect via several mechanism and play a role in the newly diagnosed as well as the relapsed/ refractory patients. In this review, we will focuse on the use of the three IMiDs （thalidomide, lenalidomide and pomalidomide） in the treatment of AL amyloidosis.