AL型肾淀粉样变患者临床病理特征分析  

作　　者：闫慧玲[1] 王宇晖[1] 俞东容[1] 

机构地区：[1]杭州市中医院,310012

出　　处：《浙江临床医学》2025年第2期276-278,282,共4页Zhejiang Clinical Medical Journal

基　　金：浙江省中医药科技计划项目(2024ZL646)。

摘　　要：目的探讨轻链型淀粉样变(light chain amyloidosis,AL)肾损害的临床及病理特点。方法回顾性分析2011年6月至2023年6月在杭州市中医院确诊为AL型肾淀粉样变患者的临床病理资料。结果研究共纳入56例患者,平均年龄为(58.8±8.9)岁。其中55.4%的患者表现为肾病综合征,26.8%的患者血肌酐升高。血免疫固定电泳阳性率为55.6%(25/45),尿免疫固定电泳阳性率为76.9%(30/39)。57.1%患者存在两个及以上脏器损害,心脏和肝脏受累较为常见。肾脏病理检查显示光镜下淀粉样物质沉积,刚果红染色呈阳性,轻链免疫荧光阳性,电镜下观察到直径约10 nm的细纤维丝。根据轻链免疫荧光结果,患者分为46例AL-λ型和10例AL-κ型。两型患者在血及尿轻链比值、血红蛋白水平、肝脏受累率、肾间质淀粉样物质阳性率方面差异具有统计学意义。结论AL型肾淀粉样变多见于中老年患者,肾病综合征为常见表现,常伴多脏器受累。肾脏病理检查对于明确诊断至关重要,AL-λ型与AL-κ型肾淀粉样变在临床表现及肾病理特征上存在差异。Objective To analyze the clinical and pathological characteristics of renal damage in light chain amyloidosis(AL).Methods A retrospective analysis was conducted on the clinical and pathological data of patients diagnosed with AL-type renal amyloidosis at Hangzhou Hospital of Traditional Chinese Medicine from June 2011 to June 2023.Results A total of 56 patients were included in the study,with an average age of(58.8±8.9)years.55.4%of the patients presented with nephrotic syndrome,and 26.8%had elevated serum creatinine.The positive rate of serum immunofixation electrophoresis was 55.6%(25/45),and the positive rate of urine immunofixation electrophoresis was 76.9%(30/39).57.1%of the patients had damage to two or more organs,with the cardiac and liver being commonly involved.Renal pathology revealed the deposition of amyloid material under light microscopy,positive Congo red staining,positive light chain immunofluorescence,and fine fibrils with a diameter of about 10 nm observed under electron microscopy.Based on the results of light chain immunofluorescence,the patients were classified into 46 cases of AL-λtype and 10 cases of AL-κtype.There were statistically significant differences in the ratio of serum and urinary light chains,hemoglobin levels,liver involvement rate,and the positive rate of renal interstitial amyloid between the two types.Conclusion AL-type renal amyloidosis is more common in middle-aged and elderly patients,often presents as nephrotic syndrome,and frequently involves multiple organs.Renal pathological examination can confirm the diagnosis,and there are differences in clinical manifestations and renal pathology between AL-λtype and AL-κtype renal amyloidosis.

关 键 词：淀粉样变性 肾疾病 免疫球蛋白轻链淀粉样变 

分 类 号：R692[医药卫生—泌尿科学]