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淀粉样变是一组原因不明的疾病的统称,其特征为异常蛋白质沉积在细胞外。它可沉积在局部或全身,侵袭任何组织,引起患处永久性功能损害,病变增大判一定程度,造成梗阻或压迫邻近器官而产生症状。治疗正在进展,着重于支持疗法,但不令人满意。将近10％的患者初诊于外科,切除局部沉积物可望治愈,外科治疗还能增加全身性淀粉样变的生存期。分类淀粉样变在显微镜下的显示大致相似,但各型沉积物的主要成分显著不同。按其主要纤丝类型及原有疾病的临床病理过程淀粉样变的分类见表1。淀粉样变纤丝成分按氨基酸顺序法测定。淀粉样轻链蛋白(AL)纤丝由完整的或分段的免疫球蛋白
Amyloidosis is a collective term for a group of unexplained diseases characterized by abnormal protein deposition outside the cell. It can be deposited in the local or systemic invasion of any tissue, causing permanent damage to the affected area, the lesion increased to a certain extent, causing obstruction or oppression of neighboring organs and symptoms. Treatment is progressing, focusing on supportive therapies, but not satisfactory. Nearly 10% of patients are newly diagnosed with surgery and resection of local deposits is expected to be cured. Surgical treatment can also increase systemic amyloidosis survival. Classification Amyloidosis shows roughly similar under a microscope, but the major components of each type of sediment are significantly different. Table 1 shows the classification of amyloidosis according to its major fibril types and the clinical and pathological course of the original disease. Amyloid fibrillation composition by amino acid sequence method. Amyloid light chain protein (AL) fibrils consist of intact or segmented immunoglobulins