摘要
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.
出处
《临床血液学杂志》
CAS
2013年第5期660-663,共4页
Journal of Clinical Hematology
基金
中国医师协会多发性骨髓瘤基金(No:20090101)
关键词
原发性系统性淀粉样变性
新药
移植
治疗进展
primary systemic amyloidosis
new drugs
transplantation
treatment progress