原发性全身性淀粉样变(AL)是一种少见疾病,具有存在淀粉样纤丝的特征。淀粉样纤丝主要在心脏、肾脏、胃肠道、周围神经和自主神经、皮肤、关节和所有器官血管内沉积。淀粉样纤丝是由免疫球蛋白轻链的一个片断所组成。轻链来源于低度增生的单克隆浆细胞。在美国从未报道过AL病死率的统计学资料,亦未见美国或其他国家有本病发病率和流行情况的报 Primary systemic amyloidosis (AL) is a rare disease characterized by the presence of amyloid fibrils. Amyloid fibrils are mainly deposited in the blood vessels of the heart, kidney, gastrointestinal tract, peripheral and autonomic nerves, skin, joints and all organs. Amyloid fibril is composed of a fragment of immunoglobulin light chain. Light chains are derived from low-proliferation monoclonal plasma cells. In the United States has never reported the statistical data on the mortality rate of AL, nor the United States or other countries reported the incidence and prevalence of this disease