肾淀粉样变的诊断与病理分型分析  

Clinicopathology, diagnosis and classification of renal amyloidosis

作　　者：任丽[1] 刘红[1] 许迅辉[1] 蒋素华[1] 方艺[1] 丁小强[1] 

机构地区：[1]复旦大学附属中山医院肾内科,上海200032

出　　处：《中华肾脏病杂志》2011年第10期730-734,共5页Chinese Journal of Nephrology

基　　金：上海市重大课题（08DZ1900602）;教育部国家“211工程”重点学科建设项目（三期）（211XK20）

摘　　要：目的分析肾淀粉样变患者肾脏及其他损伤脏器的临床病理特点，有利早期诊断肾淀粉样变。方法分析2006年至2010年在本院肾内科确诊的肾淀粉样变患者的临床和肾脏病理资料，并根据肾组织中淀粉样蛋白A、轻链蛋白K、入沉积情况进行分型。结果26例患者入选，年龄（58．54±10．07）（40—77）岁。22例（84．62％）患者曾在当地诊治0.8．24个月，其中21例临床诊断为原发性肾小球肾炎，误诊率为95．45％。肾淀粉样变患者常见临床表现为肾病综合征（17例，65．38％）、血压下降（16例，61．53％）、组织器官体积增大（8例，30．77％）、体质量下降（6例，23．08％）。56．00％（14／25例）患者血免疫固定电泳阳性。3例（11．54％）患者光镜表现不典型，刚果红染色可疑阳性，经电镜确诊为。肾淀粉样变。AL型23例（88．46％），AA型1例（3．85％），高度怀疑遗传型1例，未分型1例。结论肾淀粉样变好发于中老年患者，无特异性临床表现，误诊率高。中老年。肾病综合征患者伴血压下降、体质量下降、器官体积增大应高度怀疑本病。大多数患者伴有单克隆免疫球蛋白轻链阳性。肾组织病理检查，尤其刚果红染色和电子显微镜检查对早期诊断极为重要。免疫组织化学染色对肾淀粉样变的分型及指导治疗至关重要。Objective To clarify the clinicopathological features of renal amyloidosis in order to achieve early diagnosis and treatment. Methods Clinicopathological data of 26 biopsy- proven renal amyloidosis cases in Department of Nephrology, Zhongshan Hospital, Fudan University between 2006 and 2010 were analyzed retrospectively. Immunohistochemistry and immunofluorescence of amyloid A protein, immunoglobulin light chains such as κ, λwere performed on renal specimens for further classification. Results Age of 26 patients ranged from 40 to 77 years old, average （58.54±10.07） years. Twenty-two out of 26 patients （84.62%） were treated in local hospital before admitted to our department, and 21 patients （95.45%） were misdiagnosed as chronic primary glomerulonephritis. The prominent clinical manifestations of renal amyloidosis were nephrotic syndrome （17 cases, 65.38%）, decreased blood pressure （16 cases, 61.53%）, organ enlargement （8 cases, 30.77%） and bodyweight loss （6 cases, 23.08%）. Fourteen out of 25 patients （56.00%） were found to have monoclonal light chains in serum by immunofixation electrophoresis. Three patients with mild pathological changes who had no confirmable Congo red stain were confirmed by electron microscopy. Twenty-three （88.46%） patients were diagnosed as AL amyloidosis, one （3.85%） as AA amyloidosis, one was strongly suspected of hereditary amyloidosis,and one was undetermined. Conclusions Renal amyloidosis is frequently misdiagnosed. Middleaged and old nephrotic patients with decreased blood presure, organ enlargement and bodyweight loss may be the most helpful clues of the disease. Most patients have monoclonal light chains in serum or urine. Renal biopsy, especially electronic microscopy plays a crucial role in the early diagnosis of renal amyloidosis. Immunohistoehemistry is important for patients with renal amyloidosis in pathological classification and treatment.

关 键 词：肾疾病 淀粉样变性 病理学 临床 轻链 分型 

分 类 号：R692[医药卫生—泌尿科学]