淀粉样变性的本质是一种蛋白构象病。错误折叠的蛋白无法正常溶解,在细胞外聚集形成不溶性的纤维丝,沉积在除了脑室膜之外的任何组织,损伤组织结构并进一步导致脏器功能的受损。引起原发系统性淀粉样变性的蛋白有多种,在血液系统中以轻链沉积引起的原发系统性淀粉样变性最为常见。淀粉样变性的发病率约为0.8/10万,60~79岁为发病的高峰年龄〔1〕。其治疗方法多来源于多发性骨髓瘤的治疗方案。最近,由于一些新 The nature of amyloidosis is a protein conformation disease. Misfolded proteins fail to dissolve normally, accumulate extracellularly to form insoluble filaments, deposit on any tissue other than ventricular membranes, damage tissue structure and further lead to impaired organ function. A variety of proteins cause primary systemic amyloidosis. Primary systemic amyloidosis caused by light chain deposition in the blood system is most common. The incidence of amyloidosis is about 0.8 / 100000, 60 to 79 years of age for the incidence of the peak age 〔1〕. Most of its treatment from multiple myeloma treatment options. Recently, due to some new