病理學(xué)-肝膽胰疾病(下)

肝臟、膽道、外分泌部胰腺疾?。―iseasesoftheLiver,GallBladderandExocrinePancreas）

1ViralhepatitisAlcoholicliverdiseaseInbornerrorsofmetabolismLivercirrhosisCarcinomaofliverCholelithiasis、cholecystitisandcholangitis,carcinomaofbileductPancreatitisandcarcinomaofpancreas2ViralhepatitisAlcoholicliverdiseaseInbornerrorsofmetabolismLivercirrhosisCarcinomaofliverCholelithiasis、cholecystitisandcholangitis,carcinomaofbileductPancreatitisandcarcinomaofpancreas3肝豆?fàn)詈俗冃裕╓ilson’sDisease）13q14-21，ATP7B常染色體隱形遺傳病多為兒童及青少年膽小管上銅輸出的ATP酶失活銅離子過量肝、CNS、腎角膜kayser-Fleischer環(huán)4MicronodularcirrhosisinWilsondisease5MicronodularcirrhosisinWilsondisease6NearlyallpatientswithneurologicinvolvementdevelopeyelessionKayser-FleischerringsresultfromabuildupofcopperintheeyesandarethemostuniquesignofWilsondisease.Theyappearineacheyeasarusty-brownringaroundtheedgeoftheirisandintherimofthecornea.7M/56.Thispatientdiedfromliverfailure.Thecirrhoticnodulesareclearlyseen.Theliverisadarkbrowncolorbecauseoftheheavyirondepositionwithinthehepatocytes8910Prussianblue-stained11APrussianblueironstaindemonstratesthebluegranulesofhemosiderininhepatocytesandKupffercells.12ThePrussianblueironstain13Pancreas:Microscopically,isletcellsandstromairondepositionisblueinPrussianblue–stainedslide.Grossly,diffuseinterstitialfibrosisispresent.14血色病(hemochromatosis)常染色體隱性遺傳病發(fā)病率1/300~1/400多見于男性，約40~60歲出現(xiàn)癥狀鐵攝入正常，但鐵在胃及小腸中過量吸收致鐵鹽在肝和相關(guān)器官實(shí)質(zhì)細(xì)胞中過量沉積肝受累最早肝功能異常，進(jìn)行性門靜脈性肝硬化?肝性腦病、上消化道出血、15%HCC15繼發(fā)性含鐵血黃素沉積癥

（seconderayhemosiderosis)體內(nèi)紅細(xì)胞破壞過多溶血性貧血、重型β海洋性貧血多次輸血長期大量服用鐵劑16ViralhepatitisAlcoholicliverdiseaseInbornerrorsofmetabolismLivercirrhosisCarcinomaofliverCholelithiasis、cholecystitisandcholangitis,carcinomaofbileductPancreatitisandcarcinomaofpancreas17肝硬化

LivercirrhosisReneLa?nnecGreek

κιρρ??[kirrhós]meaningyellowish,tawny

18肝硬化

（livercirrhosis）

肝細(xì)胞彌漫性變性壞死纖維組織增生肝細(xì)胞結(jié)節(jié)狀再生反復(fù)交錯(cuò)肝臟彌漫變形、變硬血液循環(huán)改建19Grossappearance20

basicpathologicchange

纖維組織增生

假小葉（pseudolobule）原有肝臟病變21假小葉（pseudolobule）

廣泛增生的纖維組織分割包繞肝細(xì)胞（包括原有的肝小葉和再生的肝細(xì)胞結(jié)節(jié)）而形成的大小不等的肝細(xì)胞團(tuán)。假小葉內(nèi)中央靜脈可缺如、偏位或兩個(gè)以上，可以出現(xiàn)匯管區(qū)。22Histopathology23PathogenesisSchematicofstellatecellactivationandliverfibrosisincomparisontothenormalliver.24

發(fā)病機(jī)制：病因肝細(xì)胞彌漫性壞死、炎癥網(wǎng)狀纖維支架塌陷纖維組織增生分割包繞肝小葉、結(jié)節(jié)狀再生的肝細(xì)胞

假小葉

肝小葉結(jié)構(gòu)破壞肝星狀細(xì)胞-肌纖維母細(xì)胞匯管區(qū)成纖維細(xì)胞等產(chǎn)生膠原異常血管吻合血液循環(huán)途徑改建小血管堵塞（炎癥）中大血管（血栓）25

臨床病理聯(lián)系：

1.門脈高壓癥

portalhypertension

2.肝功能不全

hepaticin-sufficiencyhepaticfailure26門靜脈高壓癥肝A小葉間A門V小葉間V（匯管區(qū)）血竇中央靜脈終末門微V終末肝微A小葉下V肝V下腔V27門脈壓力增高原因1、肝血竇閉塞，竇周纖維化（竇性阻塞）282、假小葉壓迫小葉下靜脈→中央靜脈→肝血竇→門靜脈回流受阻（竇后性阻塞）門脈壓力增高原因293、肝動(dòng)脈與門靜脈間異常吻合→動(dòng)脈血流入門靜脈→門靜脈壓力↑(竇前性)門脈壓力增高原因30CausesofportalhypertensionPre-hepaticPost-hepaticIntra-hepatic

Post-hepatichepaticveinthrombosisrightheartfailureconstrictivepericarditisPre-hepaticstenosisofportalveinthrombosisIntra-hepaticSchistosomiasisseverediffusefattychangesarcoidosisTB

diffuselivercirrhosisnodularregenerativehyperplasia31

淤血性脾腫大（Congestivesplenomagely）32PortalHypertensionClinicalFeatures1、Congestivesplenomagely脾功能亢進(jìn)血液淤滯單核-巨噬細(xì)胞吞噬↑外周血細(xì)胞減少332、Ascites腹水，腹膨隆

淡黃色透明漏出液原因：（1）門靜脈壓力↑，肝竇流體靜壓↑，管壁通透性↑（2）肝臟合成蛋白↓，低蛋白血癥，血漿膠體滲透壓↓（3）肝滅活醛固酮、抗利尿激素功能↓，水鈉潴留34353.PortosystemicShunts

食管下段靜脈叢臍周及腹壁靜脈叢直腸靜脈叢歪門邪道奇靜脈上腔靜脈下腔靜脈髂內(nèi)靜脈3637臍周及腹壁靜脈叢曲張（海蛇頭,CaputMedusae）38Seenhereistheanusandperianalregionwithprominentprolapsedtruehemorrhoids.Chronicconstipation,chronicdiarrhea,pregnancy,andportalhypertensionenhancehemorrhoidformation.Hemorrhoidscanitchandbleedslowly(usuallybrightredblood,duringdefaecation).

394、胃腸瘀血、水腫消化、吸收功能↓

腹脹食欲不振40肝功能不全

hepaticinsufficiencyBiochemicalabnormalitiesClinicalmanifestationEstrogen:elevatedSpiderangiomas(uppertrunk),Palmar

erythema,Hypogonadism(atrophictesticles),GynecomastiaSerumalbumin:lowAscitesCoagulativefactors:decreasedProthrombintime:prolongedTendencytohemorrhageBilirubin:elevatedJaundice,pruritusTransaminases(ASTandALT):elevatedAnorexia,nausea,generalmalaiseHyperammonemiaHepaticencephalopathy41黃疸（jaundice）42男子乳腺發(fā)育

Gynecomastiaduetoalcoholiccirrhosis-A32yearoldmalepatientwithnormalsecondarysexcharacteristics,notesticularmass,nohistoryofdrugingestion,nootherendocrineabnormalitiesandanormalneurologicalexamination.

Nevertheless,hehadahistoryofmorethan15yearsoflargeamountsofalcoholintakeandaliverbiopsyconfirmalcoholiccirrhosis.

43肝掌Palmarerythema蜘蛛狀痣Spiderangiomas(uppertrunk)44出血傾向(Spontaneousbleeding)45

Clinicalmanifestation46TheclassificationofcirrhosisAccordingtothegrossmorphologyMicronodular（<3mm）

Macronodular（>3mm）MixednodularIncompletedseparated47Thedescriptivetermsshouldnotbeusedasprimaryclassification48TheclassificationofcirrhosisAccordingtothecausesAlcoholicViralhepatitisCardiacBiliaryParasiticPigmentalCryptogenic49M/39.withhepatitisBinfection.Theliverisgreatlyenlargedandweighted1720g.Itssurfaceiscoarselynodular.50Hereisanexampleofalcoholiccirrhosis.Notethattheliveralsohasayellowishhue,indicatingthatfattychangeispresent.51SchistosomiasisCirrhosis5253膽汁性肝硬化

Biliarycirrhosis原發(fā)性(少見）

90%女性自身免疫性損傷肝內(nèi)小膽管慢性非化

膿性破壞性膽管炎慢性進(jìn)行性膽汁淤積不全分割型5455繼發(fā)性肝外膽管堵塞、膽道上行性感染56NutmegliverCardiaccirrhosis57TheclassificationofcirrhosisIntegratedclassificatonPortal(micronodular）

Postnecrotic(macronodular

ormixed）Biliary(Incompletedseparated)CongestiveParasitic…581、病毒性肝炎：HBVHCV（我國最常見的原因）2、慢性酒精中毒：（歐、美國家最常見原因）3、營養(yǎng)缺乏：膽堿、蛋氨酸；合成磷脂障礙

脂肪肝4、化學(xué)毒物：殺蟲劑、CCl4等病因門脈性肝硬化（portalcirrhosis）59壞死后性肝硬化（postnecroticcirrhosis）肝實(shí)質(zhì)發(fā)生大片壞死的基礎(chǔ)上形成的較嚴(yán)重的病毒性肝炎

亞急性重型肝炎重度慢性肝炎+壞死嚴(yán)重藥物及化學(xué)物質(zhì)中毒6061病理特點(diǎn)：大體：體積小，重量輕，質(zhì)硬，表面及切面結(jié)節(jié)狀(＞1cm)，纖維間隔厚，厚薄不均鏡下：假小葉形態(tài)懸殊肝細(xì)胞不同程度變性、壞死寬大的纖維間隔膽管增生和炎細(xì)胞浸潤顯著postnecroticcirrhosis62臨床肝功能障礙出現(xiàn)早，重

門脈高壓出現(xiàn)晚，輕惡變率高postnecroticcirrhosis63SummaryIncirrhosistheliverisdiffuselyreplacedbynodulesofhepatocytesseparatedbyfibrosisThemostcommoncausesofcirrhosisarediseasesofalcoholabuseandviralhepatitisCirrhosisisbestclassifiedaccordingtothecauseofliverdamage64Summary

ThemostcommonanddangerouscomplicationsofcirrhosisFatalbleedingofloweresophagealplexusHepaticcoma(hepaticencephalopathy)Hepatocellularcarcinoma,especiallyforthecasefromchronichepatitisBandC65ViralhepatitisAlcoholicliverdiseaseInbornerrorsofmetabolismLivercirrhosisCarcinomaofliverCholelithiasis、cholecystitisandcholangitis,carcinomaofbileductPancreatitisandcarcinomaofpancreas66原發(fā)性肝癌

(primarycarcinomaof

theliver)

原發(fā)于肝細(xì)胞或肝內(nèi)膽管上皮細(xì)胞的惡性腫瘤。67肝癌發(fā)病率大國，發(fā)病率占全球45%高發(fā)年齡40~50歲起病隱匿（40%診斷時(shí)沒有癥狀）確診時(shí)多數(shù)已達(dá)到中晚期5年生存率2%-16%病因病毒性肝炎肝硬化黃曲霉素68高危人群乙肝表面抗原陽性者慢性肝炎患者有肝癌家族史早期發(fā)現(xiàn)肝癌——定期體檢每半年做一次肝功能、AFP、B超69病變?nèi)庋垲愋停?/p>

早期肝癌（小肝癌）中晚期肝癌：巨塊型

多結(jié)節(jié)型彌漫型

多合并肝硬化70MassivetypeofHCCina17-year-oldpatientwithoutcirrhosis71MassivetypeofHCC72Thelivershowinganunifocalmassiveneoplasmwithcirrhosis;somesatellitetumornodulesisdirectlyadjacent.

73MutifocaltypeofHCC74DiffuselyinfiltrativetypeofHCC75HCCwithintrahepaticmetastasis76tumorthrombosis77Adenocarcinomaarisingfromthebileductsintheportahepatis.F/78.Thepatientdiedfromtheeffectofobstructivejaundice.cholangiocarcinoma787980HCC81cholangiocarcinoma82Combinedhepatocellularandcholangiocarcinoma83肝臟其他常見腫瘤

1.原發(fā)

2.

繼發(fā)（轉(zhuǎn)移）更常見良性惡性海綿狀血管瘤肝細(xì)胞腺瘤等肝母細(xì)胞瘤血管肉瘤等84Cavernoushaemangiomaintheliver.Thiswasanincidentalfinding.Occasionallythesehaemangiomasmaybleed,butmostlytheyareasymptomatic.85Mostliver-celladenomasariseinwomenofchild-bearingage,usuallyafterprolongeduseoforalcontraceptives.Subcapsularadenomasareatriskforrupture.86metastaticcarcinomasfromthestomach87MetastaticcarcinomainliverThelivermaybeinvolvedbysecondarytumors,presentingmultiplehepaticmetastases.88ViralhepatitisAlcoholicliverdiseaseInbornerrorsofmetabolismLivercirrhosisCarcinomaofliverCholelithiasis、cholecystitisandcholangitis,carcinomaofbileductPancreatitisandcarcinomaofpancreas89正常膽囊（normalgallbladder）90pigmentstonesarepredisposedbyincreasedhepaticsecretionofbilirubin.

cholesterolstonesarepredisposedbychangesincholesterolsolubilityinbile.

919293Chroniccholecystitis.F/48.Thegallbladdershowsthefeaturesofchroniccholecystitisanditispackedwithsmallmultifacetedmixedgallstones.94Acutesuperimposedonchroniccholecystitis.F/38.Thegallbladderisdistended.Bothserosalandmucosalsurfacesarereddenedandinflamed.Thewallofthegallbladderisthickenedandthereareafewmixedcalculiwithinit.95Cholesterolosis.F/36.Thegallbladdershowschroniccholecystitis.Therearemultipleyellowspotsonitsmucosalsurface.Thisisduetotheaccumulationoflipidinthelaminapropria.9697肝內(nèi)膽管結(jié)石98膽道腫瘤膽囊和肝外膽管癌

(carcinomaofthegallbladderandextrahepaticbileducts)

膽囊和肝外膽管上皮細(xì)胞發(fā)生的惡性腫瘤

99Carcinomaofthegallbladder.Adenocarcinomaofthegallbladderisseenasaraisedareainfundus.Mosttumorshaveinvadedthroughthewallatthetimeofdiagnosis.100101102胰腺外分泌部

（Exocrinepancreas）胰腺實(shí)質(zhì)由外分泌部和內(nèi)分泌部組成外分泌部占90%，分泌胰液，經(jīng)導(dǎo)管排入十二指腸，有重要的化學(xué)性消化作用急性胰腺炎（Acutepancreatitis）慢性胰腺炎（Chronicpancreatitis）胰腺假性囊腫（Pseudocystofthepancreas）腫瘤（Tumors）103Normalcommonbileductandpancreaticduct104急性水腫性（間質(zhì)性）胰腺炎

acuteedemouspancreatitis暴飲暴食（酗酒）；膽道結(jié)石間質(zhì)充血水腫炎細(xì)胞浸潤（中性粒細(xì)胞和單核細(xì)胞）可局灶性脂肪壞死預(yù)后好105Acutehemorrhagicpancreatitis106Hemorrhageandnecrosis107Lipasereleasedfromthepancreaticacinicausesthedevelopmentoffocioffatnecrosis.Thisisseenaswhitespots.108109Histologicallythesespotsarecomposedofnecroticadiposetissue,withadjacentreactiveinflammation.110特點(diǎn)：發(fā)病急，病情重病理：胰腺腫大，結(jié)構(gòu)模糊；