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1、萬(wàn)方數(shù)據(jù)生坐盟業(yè)亟垂查2QQ!生!旦筮12鲞塑!趔￡b也』d業(yè)!墨!：△!趕!!Z業(yè)!：!生!!：№：!·腎臟疾病病理專(zhuān)題論著‘輕鏈腎病和輕鏈型淀粉樣變腎臟損害的臨床和病理陳楠任紅史浩潘曉霞張文俞海瑾陳曉農(nóng)郝翠蘭【摘要】目的總結(jié)輕鏈腎病(LCN)和輕鏈型淀粉樣變(AL)腎臟損害臨床和病理改變。方法回顧性分析1990～2000年25例AL及6例LCN患者臨床、實(shí)驗(yàn)室及組織病理檢查結(jié)果。結(jié)果首發(fā)癥狀多為水腫。96％AL和66．7％LCN出現(xiàn)蛋白尿．其中75％為腎病綜合征(Ns)。腎功能不全見(jiàn)于56％^L和83，3％LCN。約50％患者腎臟
2、體積增大。腎小管功能不全發(fā)生率較高(>60％)。腎外改變以貧血和肝脾腫大最為常見(jiàn)。LCN光鏡主要呈腎小球系膜結(jié)節(jié)性硬化(4例)．6例LCN標(biāo)本均見(jiàn)單克隆輕鏈(x4例．x2例)在腎小管基底膜發(fā)部分腎小球內(nèi)沉積。所有AL標(biāo)本見(jiàn)腎小球、小管問(wèn)質(zhì)及小葉問(wèn)動(dòng)脈淀粉樣物質(zhì)沉積．剛果紅染色(十)，偏光顯微鏡下呈蘋(píng)果綠雙折光．高錳酸鉀預(yù)處理試驗(yàn)不褪色(10例)．免疫組化AA蛋白(一)。6例AL伴多發(fā)性骨髓瘤，骨髓漿細(xì)胞>15％。2例LCN合井異常增高lgM單株峰'病理亦支持巨球蛋白血癥診斷。結(jié)論AL和LCN并非少見(jiàn)病，臨床以蛋白尿和Ns最常見(jiàn)，腎功能
3、受累發(fā)生率高，腎外表現(xiàn)多見(jiàn)。病理檢查尤其特殊病理檢查對(duì)診斷和鑒別有重要作用?！娟P(guān)鍵詞】淀粉樣變；免疫球蛋白類(lèi)，輕鏈；腎?。慌R床；病理ClinicalandpathologicalmanlfeslationsoflightchainnephropathyandprimaryamyloidosisCHENNan．RENltong,Silltfao，etalDepartraeraofNe#rology．RaijlnHospital,ShanghaiSecondMedicalUniversity，Shanghai200025．China【Ab
4、stract】ObjectiveToinvestigatetheclinicalandpathologicalmanifestations“l(fā)ightchainnephropathy(LCN)andprimaryamyloidosis(AL)．MethodsTwenty—fivepatientswithAL《19male,6female)and6withLCN(3male，3female)werehospitalizedfrom1990to2000．TheirclinicalfindingB，laboratoryresultsandp
5、athol晤∞】changeswei,PⅢiewed．ResultsMostofthecaseswithALorLcNpresentededemaatfirstonseLProteinuriawasfoundin96％ofALpatien．sand66．7％ofLCNpatients．ofwhich75％w￡l,smephrtaicsyndrome56％ofALand83．3％ofLCNhadrenalinsufficiency．Enla曜edkidneywasfoundinabont50％ofallthepatients．Renal
6、tubulninterstitialimpairmentoccurredfrequendy(>60％)．Anemiaandhepatosplenomegalywerernostcommonextm·renalmanifestationsUnderlightmicroscopy．LCNch洲tedsLicallvshowedmermngialnodularsclerosis．LinearstainingofmonoclonalKorxlightchainalongthetubularbasementmembraneandinthenod
7、aleswasfoundinallLCNsamples．ALshowedgiomemlar,tubulointerstitialandbloodvesseldepositionofamorphousmaterial．PositivecongoredstainandthediagnosticapplegreenbirefringeneeunderpolarizedlightwereobservedinALsamples，meanwhileimmunostainingofserumamyloidAproteinwasnegative．Si
8、xALpatientswereaccompaniedhymultiplemyelomaand2LCNaccompaniedbyWaldenstrom7smaetogiobulinemia(WM)withobviously
