Thomsen J S, Horn T, Schlichting P
Patologisk-anatomisk institut, Amtssygehuset i Herlev.
Ugeskr Laeger. 1999 May 24;161(21):3079-83.
Amyloidosis is a heterogenous group of diseases, all characterized by extracellular deposition of amyloid either systemically or localized. Of wellknown diseases are Alzheimer's dementia, AL-amyloidosis (e.g. in multiple myeloma) and AA-amyloidosis (e.g. in rheumatoid arthritis). Amyloid is composed of three components of which the fibrillary component is the basis of amyloid classification. Many types of amyloid have a systemic distribution and give rise to varying symptoms. The diagnosis is based on biopsy, preferably of abdominal subcutis. The prognosis is poor, however, recent investigations on the three-dimensional structure of the P-component provide hope for future therapy.
淀粉样变性是一组异质性疾病,其共同特征是淀粉样蛋白在全身或局部细胞外沉积。常见的疾病有阿尔茨海默病性痴呆、AL型淀粉样变性(如在多发性骨髓瘤中)和AA型淀粉样变性(如在类风湿关节炎中)。淀粉样蛋白由三种成分组成,其中纤维状成分是淀粉样蛋白分类的基础。许多类型的淀粉样蛋白具有全身分布并引起不同症状。诊断基于活检,最好是腹部皮下组织活检。预后较差,然而,最近对P成分三维结构的研究为未来治疗带来了希望。