新药和移植治疗原发性系统性淀粉样变性的进展

The treatment progress of new drugs or transplant on primary systemic amyloidosis

原发性系统性淀粉样变性（AL）是一种少见病，发病率为（5～12）／1000000。其本质是一种蛋白构象病，是免疫球蛋白轻链发生淀粉样变性并在脏器直接沉积引发的脏器功能改变的疾病，

Summary Primary systemic amyloidosis （AL） is not a common disease, but usually involving multiple organs and misdiagnosed, early identification can improve the prognosis. With the development of new targeted drugs such as bortezomib, thalidomide,lenalidomide and so on, autologous transplantation in the status of ALif pretreatment of adding new drugs can improve the prognosis, whether there is need to use drugs after transplantation to maintain therapy or not and its curative effect, are the questions which clinicians should explore. This review will summarize the current progress of these aspects.