Browning M J, Banks R A, Tribe C R, Hollingworth P, Kingswood C, Mackenzie J C, Bacon P A
Q J Med. 1985 Mar;54(215):213-27.
The aetiology, clinical course and affected organs were studied in 124 patients with acquired systemic amyloidosis and seven patients with organ-limited amyloid deposits. Seventy-five patients had reactive systemic AA amyloidosis, which was associated with rheumatic disease in 55 and with chronic infection in 13 cases. Forty-nine patients had systemic AL amyloidosis. Thirteen of these cases were associated with myelomatosis and 11 with non-malignant immunocyte dyscrasias. In 25 patients with systemic AL disease no immunocyte dyscrasia was identified. Renal involvement dominated the clinical course of both forms of systemic amyloidosis, and renal failure was the most common cause of death. Gastrointestinal disturbance and hepatosplenomegaly were found in both AA and AL disease, although differences were noted in the distribution of amyloid protein within rectal biopsies. Amyloid cardiomyopathy, neuropathy and macroglossia were present in patients with AL amyloidosis only. These clinical patterns were reflected by tissue distribution at necropsy in 67 patients.
对124例获得性系统性淀粉样变性患者和7例器官局限性淀粉样沉积物患者的病因、临床病程及受累器官进行了研究。75例患者患有反应性系统性AA淀粉样变性，其中55例与风湿性疾病相关，13例与慢性感染相关。49例患者患有系统性AL淀粉样变性。其中13例与骨髓瘤相关，11例与非恶性免疫细胞异常增生相关。25例系统性AL疾病患者未发现免疫细胞异常增生。肾脏受累在两种形式的系统性淀粉样变性临床病程中占主导地位，肾衰竭是最常见的死亡原因。AA和AL疾病均发现胃肠道紊乱和肝脾肿大，尽管直肠活检中淀粉样蛋白的分布存在差异。淀粉样心肌病仅见于AL淀粉样变性患者，神经病变和巨舌症也是如此。67例患者尸检时的组织分布反映了这些临床模式。
