肾淀粉样变性临床病理学分析

Clinicopathologic analysis of renal amyloidosis: An analysis of 15 cases

目的探讨肾脏淀粉样变性的临床病理学特点。方法对15例经病理诊断为肾淀粉样变性患者的临床资料、免疫荧光、组织化学及光镜检验结果进行回顾性分析和研究。结果疾病初期通常表现为肾病综合征,随着疾病的发展,临床可出现肾功能不全和肾衰竭。原发性肾淀粉样变性11例,继发性肾淀粉样变性4例。光镜下淀粉样物质可沉积于肾小球系膜、基底膜、血管壁及肾间质,这些受累的区域无细胞或细胞过少。随着病程的发展,肾小球可以完全被淀粉样物质替代(3/15)。淀粉样沉积物刚果红染色阳性(15/15)。结论肾脏淀粉样变性的病因是多方面的,其诊断依赖于组织病理学和组织化学,并须结合其临床资料。

Objective To investigate the clinicopathologic characteristics of renal amyloidosis. Methods The clinicopathologic data were collected from 15 cases of renal amyloidosis confirmed by pathology. They were retrospectively analyzed by light microscopy,histochemistry and immunofluorescense. Results Initially the renal manifestation was usually the nephrotic syndrome.As the disease progressed,renal insufficiency and renal failure appeared.Primary kidney amyloidosis was found in 11 cases,and secondary kidney amyloidosis in 4 cases. Histologically, amyloid material was deposited in the glomerular mesangia, glomerular basement membranes,vessel walls and interstitium.The areas affected appeared acellular or hypocellular.In advanced stages,the glomeruli were completely replaced with amyloid material and appeared acellular （3/15）. Amyloid deposits were positive for Congo Red （15/15）.Concluslons The etiologies of amyloidosis are barious.The diagnosis of the disease depends on its histopathology and histochemistry examination. Its clinical manifestation should also be taken into account.