轻链肾病和轻链型淀粉样变肾脏损害的临床和病理VIP专享VIP免费

生坐盟业亟垂查2QQ!生!旦筮12鲞塑!趔￡b也』d业!墨!：△!赶!!Z业!：!生!!：№：!·肾脏疾病病理专题论著‘轻链肾病和轻链型淀粉样变肾脏损害的临床和病理陈楠任红史浩潘晓霞张文俞海瑾陈晓农郝翠兰【摘要】目的总结轻链肾病(LCN)和轻链型淀粉样变(AL)肾脏损害临床和病理改变。方法回顾性分析1990～2000年25例AL及6例LCN患者临床、实验室及组织病理检查结果。结果首发症状多为水肿。96％AL和66．7％LCN出现蛋白尿．其中75％为肾病综合征(Ns)。肾功能不全见于56％^L和83，3％LCN。约50％患者肾脏体积增大。肾小管功能不全发生率较高(>60％)。肾外改变以贫血和肝脾肿大最为常见。LCN光镜主要呈肾小球系膜结节性硬化(4例)．6例LCN标本均见单克隆轻链(x4例．x2例)在肾小管基底膜发部分肾小球内沉积。所有AL标本见肾小球、小管问质及小叶问动脉淀粉样物质沉积．刚果红染色(十)，偏光显微镜下呈苹果绿双折光．高锰酸钾预处理试验不褪色(10例)．免疫组化AA蛋白(一)。6例AL伴多发性骨髓瘤，骨髓浆细胞>15％。2例LCN合井异常增高lgM单株峰'病理亦支持巨球蛋白血症诊断。结论AL和LCN并非少见病，临床以蛋白尿和Ns最常见，肾功能受累发生率高，肾外表现多见。病理检查尤其特殊病理检查对诊断和鉴别有重要作用。【关键词】淀粉样变；免疫球蛋白类，轻链；肾病；临床；病理ClinicalandpathologicalmanlfeslationsoflightchainnephropathyandprimaryamyloidosisCHENNan．RENltong,Silltfao，etalDepartraeraofNe#rology．RaijlnHospital,ShanghaiSecondMedicalUniversity，Shanghai200025．China【Abstract】ObjectiveToinvestigatetheclinicalandpathologicalmanifestations“lightchainnephropathy(LCN)andprimaryamyloidosis(AL)．MethodsTwenty—fivepatientswithAL《19male,6female)and6withLCN(3male，3female)werehospitalizedfrom1990to2000．TheirclinicalfindingB，laboratoryresultsandpathol晤∞】changeswei,PⅢiewed．ResultsMostofthecaseswithALorLcNpresentededemaatfirstonseLProteinuriawasfoundin96％ofALpatien．sand66．7％ofLCNpatients．ofwhich75％w￡l,smephrtaicsyndrome56％ofALand83．3％ofLCNhadrenalinsufficiency．Enla曜edkidneywasfoundinabont50％ofallthepatients．Renaltubulninterstitialimpairmentoccurredfrequendy(>60％)．Anemiaandhepatosplenomegalywerernostcommonextm·renalmanifestationsUnderlightmicroscopy．LCNch洲tedsLicallvshowedmermngialnodularsclerosis．LinearstainingofmonoclonalKorxlightchainalongthetubularbasementmembraneandinthenodaleswasfoundinallLCNsamples．ALshowedgiomemlar,tubulointerstitialandbloodvesseldepositionofamorphousmaterial．PositivecongoredstainandthediagnosticapplegreenbirefringeneeunderpolarizedlightwereobservedinALsamples，meanwhileimmunostainingofserumamyloidAproteinwasnegative．SixALpatientswereaccompaniedhymultiplemyelomaand2LCNaccompaniedbyWaldenstrom7smaetogiobulinemia(WM)withobviouslyelevated∞mmIgM．CondusionsALandLCNarenotrarediseasses．alostCOlfnulonclinicalfeaturesareproteinurlaandnephrtaiesyndrome．Manypatientshavevaryingdegrees0frenalimpairmentExtra-renalsymptomsaremultiplePathologicalexaminationprovidesimportantindicationfordiagnosisanddifferentialdiagnosisLKeywordsJAmyloidosis；lmmunoglobulin，lightchain；Nephropathy；Clinic；Pathology轻链型淀粉样变(AL)、轻链肾病(LCN)均属单克隆免疫球蛋白病，由淋巴、浆细胞异常增生引起．大量恶性增生的单克隆免疫球蛋白及其轻链产生一系列病理损害及临床症状，肾脏为首个或最常见的什1昔单位：200025上海第二医科大学附届瑞金医院肾内科受累器官[J41。我们从临床和病理总结了本科25例AL和6例LCN患者肾脏损害情况。对象和方法一、病例选择1990～2000年我科收治经临床病理诊断为AL万方数据214土生堑蛙亟盘查2盟!堑!旦翌!!整茎±塑曼!也』型!吐苎!：6蛆!璺垫Q!，!吐!!：盐：±25例(台...