病理学-肝胆胰疾病(下)

肝脏、胆道、外分泌部胰腺疾病（DiseasesoftheLiver,GallBladderandExocrinePancreas）

1ViralhepatitisAlcoholicliverdiseaseInbornerrorsofmetabolismLivercirrhosisCarcinomaofliverCholelithiasis、cholecystitisandcholangitis,carcinomaofbileductPancreatitisandcarcinomaofpancreas2ViralhepatitisAlcoholicliverdiseaseInbornerrorsofmetabolismLivercirrhosisCarcinomaofliverCholelithiasis、cholecystitisandcholangitis,carcinomaofbileductPancreatitisandcarcinomaofpancreas3肝豆状核变性（Wilson’sDisease）13q14-21，ATP7B常染色体隐形遗传病多为儿童及青少年胆小管上铜输出的ATP酶失活铜离子过量肝、CNS、肾角膜kayser-Fleischer环4MicronodularcirrhosisinWilsondisease5MicronodularcirrhosisinWilsondisease6NearlyallpatientswithneurologicinvolvementdevelopeyelessionKayser-FleischerringsresultfromabuildupofcopperintheeyesandarethemostuniquesignofWilsondisease.Theyappearineacheyeasarusty-brownringaroundtheedgeoftheirisandintherimofthecornea.7M/56.Thispatientdiedfromliverfailure.Thecirrhoticnodulesareclearlyseen.Theliverisadarkbrowncolorbecauseoftheheavyirondepositionwithinthehepatocytes8910Prussianblue-stained11APrussianblueironstaindemonstratesthebluegranulesofhemosiderininhepatocytesandKupffercells.12ThePrussianblueironstain13Pancreas:Microscopically,isletcellsandstromairondepositionisblueinPrussianblue–stainedslide.Grossly,diffuseinterstitialfibrosisispresent.14血色病(hemochromatosis)常染色体隐性遗传病发病率1/300~1/400多见于男性，约40~60岁出现症状铁摄入正常，但铁在胃及小肠中过量吸收致铁盐在肝和相关器官实质细胞中过量沉积肝受累最早肝功能异常，进行性门静脉性肝硬化¼肝性脑病、上消化道出血、15%HCC15继发性含铁血黄素沉积症

（seconderayhemosiderosis)体内红细胞破坏过多溶血性贫血、重型β海洋性贫血多次输血长期大量服用铁剂16ViralhepatitisAlcoholicliverdiseaseInbornerrorsofmetabolismLivercirrhosisCarcinomaofliverCholelithiasis、cholecystitisandcholangitis,carcinomaofbileductPancreatitisandcarcinomaofpancreas17肝硬化

LivercirrhosisReneLaënnecGreek

κιρρός[kirrhós]meaningyellowish,tawny

18肝硬化

（livercirrhosis）

肝细胞弥漫性变性坏死纤维组织增生肝细胞结节状再生反复交错肝脏弥漫变形、变硬血液循环改建19Grossappearance20

basicpathologicchange

纤维组织增生

假小叶（pseudolobule）原有肝脏病变21假小叶（pseudolobule）

广泛增生的纤维组织分割包绕肝细胞（包括原有的肝小叶和再生的肝细胞结节）而形成的大小不等的肝细胞团。假小叶内中央静脉可缺如、偏位或两个以上，可以出现汇管区。22Histopathology23PathogenesisSchematicofstellatecellactivationandliverfibrosisincomparisontothenormalliver.24

发病机制：病因肝细胞弥漫性坏死、炎症网状纤维支架塌陷纤维组织增生分割包绕肝小叶、结节状再生的肝细胞

假小叶

肝小叶结构破坏肝星状细胞-肌纤维母细胞汇管区成纤维细胞等产生胶原异常血管吻合血液循环途径改建小血管堵塞（炎症）中大血管（血栓）25

临床病理联系：

1.门脉高压症

portalhypertension

2.肝功能不全

hepaticin-sufficiencyhepaticfailure26门静脉高压症肝A小叶间A门V小叶间V（汇管区）血窦中央静脉终末门微V终末肝微A小叶下V肝V下腔V27门脉压力增高原因1、肝血窦闭塞，窦周纤维化（窦性阻塞）282、假小叶压迫小叶下静脉→中央静脉→肝血窦→门静脉回流受阻（窦后性阻塞）门脉压力增高原因293、肝动脉与门静脉间异常吻合→动脉血流入门静脉→门静脉压力↑(窦前性)门脉压力增高原因30CausesofportalhypertensionPre-hepaticPost-hepaticIntra-hepatic

Post-hepatichepaticveinthrombosisrightheartfailureconstrictivepericarditisPre-hepaticstenosisofportalveinthrombosisIntra-hepaticSchistosomiasisseverediffusefattychangesarcoidosisTB

diffuselivercirrhosisnodularregenerativehyperplasia31

淤血性脾肿大（Congestivesplenomagely）32PortalHypertensionClinicalFeatures1、Congestivesplenomagely脾功能亢进血液淤滞单核-巨噬细胞吞噬↑外周血细胞减少332、Ascites腹水，腹膨隆

淡黄色透明漏出液原因：（1）门静脉压力↑，肝窦流体静压↑，管壁通透性↑（2）肝脏合成蛋白↓，低蛋白血症，血浆胶体渗透压↓（3）肝灭活醛固酮、抗利尿激素功能↓，水钠潴留34353.PortosystemicShunts

食管下段静脉丛脐周及腹壁静脉丛直肠静脉丛歪门邪道奇静脉上腔静脉下腔静脉髂内静脉3637脐周及腹壁静脉丛曲张（海蛇头,CaputMedusae）38Seenhereistheanusandperianalregionwithprominentprolapsedtruehemorrhoids.Chronicconstipation,chronicdiarrhea,pregnancy,andportalhypertensionenhancehemorrhoidformation.Hemorrhoidscanitchandbleedslowly(usuallybrightredblood,duringdefaecation).

394、胃肠瘀血、水肿消化、吸收功能↓

腹胀食欲不振40肝功能不全

hepaticinsufficiencyBiochemicalabnormalitiesClinicalmanifestationEstrogen:elevatedSpiderangiomas(uppertrunk),Palmar

erythema,Hypogonadism(atrophictesticles),GynecomastiaSerumalbumin:lowAscitesCoagulativefactors:decreasedProthrombintime:prolongedTendencytohemorrhageBilirubin:elevatedJaundice,pruritusTransaminases(ASTandALT):elevatedAnorexia,nausea,generalmalaiseHyperammonemiaHepaticencephalopathy41黄疸（jaundice）42男子乳腺发育

Gynecomastiaduetoalcoholiccirrhosis-A32yearoldmalepatientwithnormalsecondarysexcharacteristics,notesticularmass,nohistoryofdrugingestion,nootherendocrineabnormalitiesandanormalneurologicalexamination.

Nevertheless,hehadahistoryofmorethan15yearsoflargeamountsofalcoholintakeandaliverbiopsyconfirmalcoholiccirrhosis.

43肝掌Palmarerythema蜘蛛状痣Spiderangiomas(uppertrunk)44出血倾向(Spontaneousbleeding)45

Clinicalmanifestation46TheclassificationofcirrhosisAccordingtothegrossmorphologyMicronodular（<3mm）

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

Biliarycirrhosis原发性(少见）

90%女性自身免疫性损伤肝内小胆管慢性非化

脓性破坏性胆管炎慢性进行性胆汁淤积不全分割型5455继发性肝外胆管堵塞、胆道上行性感染56NutmegliverCardiaccirrhosis57TheclassificationofcirrhosisIntegratedclassificatonPortal(micronodular）

Postnecrotic(macronodular

ormixed）Biliary(Incompletedseparated)CongestiveParasitic…581、病毒性肝炎：HBVHCV（我国最常见的原因）2、慢性酒精中毒：（欧、美国家最常见原因）3、营养缺乏：胆碱、蛋氨酸；合成磷脂障碍

脂肪肝4、化学毒物：杀虫剂、CCl4等病因门脉性肝硬化（portalcirrhosis）59坏死后性肝硬化（postnecroticcirrhosis）肝实质发生大片坏死的基础上形成的较严重的病毒性肝炎

亚急性重型肝炎重度慢性肝炎+坏死严重药物及化学物质中毒6061病理特点：大体：体积小，重量轻，质硬，表面及切面结节状(＞1cm)，纤维间隔厚，厚薄不均镜下：假小叶形态悬殊肝细胞不同程度变性、坏死宽大的纤维间隔胆管增生和炎细胞浸润显著postnecroticcirrhosis62临床肝功能障碍出现早，重

门脉高压出现晚，轻恶变率高postnecroticcirrhosis63SummaryIncirrhosistheliverisdiffuselyreplacedbynodulesofhepatocytesseparatedbyfibrosisThemostcommoncausesofcirrhosisarediseasesofalcoholabuseandviralhepatitisCirrhosisisbestclassifiedaccordingtothecauseofliverdamage64Summary

ThemostcommonanddangerouscomplicationsofcirrhosisFatalbleedingofloweresophagealplexusHepaticcoma(hepaticencephalopathy)Hepatocellularcarcinoma,especiallyforthecasefromchronichepatitisBandC65ViralhepatitisAlcoholicliverdiseaseInbornerrorsofmetabolismLivercirrhosisCarcinomaofliverCholelithiasis、cholecystitisandcholangitis,carcinomaofbileductPancreatitisandcarcinomaofpancreas66原发性肝癌

(primarycarcinomaof

theliver)

原发于肝细胞或肝内胆管上皮细胞的恶性肿瘤。67肝癌发病率大国，发病率占全球45%高发年龄40~50岁起病隐匿（40%诊断时没有症状）确诊时多数已达到中晚期5年生存率2%-16%病因病毒性肝炎肝硬化黄曲霉素68高危人群乙肝表面抗原阳性者慢性肝炎患者有肝癌家族史早期发现肝癌——定期体检每半年做一次肝功能、AFP、B超69病变肉眼类型：

早期肝癌（小肝癌）中晚期肝癌：巨块型

多结节型弥漫型

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

73MutifocaltypeofHCC74DiffuselyinfiltrativetypeofHCC75HCCwithintrahepaticmetastasis76tumorthrombosis77Adenocarcinomaarisingfromthebileductsintheportahepatis.F/78.Thepatientdiedfromtheeffectofobstructivejaundice.cholangiocarcinoma787980HCC81cholangiocarcinoma82Combinedhepatocellularandcholangiocarcinoma83肝脏其他常见肿瘤

1.原发

2.

继发（转移）更常见良性恶性海绵状血管瘤肝细胞腺瘤等肝母细胞瘤血管肉瘤等84Cavernoushaemangiomaintheliver.Thiswasanincidentalfinding.Occasionallythesehaemangiomasmaybleed,butmostlytheyareasymptomatic.85Mostliver-celladenomasariseinwomenofchild-bearingage,usuallyafterprolongeduseoforalcontraceptives.Subcapsularadenomasareatriskforrupture.86metastaticcarcinomasfromthestomach87MetastaticcarcinomainliverThelivermaybeinvolvedbysecondarytumors,presentingmultiplehepaticmetastases.88ViralhepatitisAlcoholicliverdiseaseInbornerrorsofmetabolismLivercirrhosisCarcinomaofliverCholelithiasis、cholecystitisandcholangitis,carcinomaofbileductPancreatitisandcarcinomaofpancreas89正常胆囊（normalgallbladder）90pigmentstonesarepredisposedbyincreasedhepaticsecretionofbilirubin.

cholesterolstonesarepredisposedbychangesincholesterolsolubilityinbile.

919293Chroniccholecystitis.F/48.Thegallbladdershowsthefeaturesofchroniccholecystitisanditispackedwithsmallmultifacetedmixedgallstones.94Acutesuperimposedonchroniccholecystitis.F/38.Thegallbladderisdistended.Bothserosalandmucosalsurfacesarereddenedandinflamed.Thewallofthegallbladderisthickenedandthereareafewmixedcalculiwithinit.95Cholesterolosis.F/36.Thegallbladdershowschroniccholecystitis.Therearemultipleyellowspotsonitsmucosalsurface.Thisisduetotheaccumulationoflipidinthelaminapropria.9697肝内胆管结石98胆道肿瘤胆囊和肝外胆管癌

(carcinomaofthegallbladderandextrahepaticbileducts)

胆囊和肝外胆管上皮细胞发生的恶性肿瘤

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

（Exocrinepancreas）胰腺实质由外分泌部和内分泌部组成外分泌部占90%，分泌胰液，经导管排入十二指肠，有重要的化学性消化作用急性胰腺炎（Acutepancreatitis）慢性胰腺炎（Chronicpancreatitis）胰腺假性囊肿（Pseudocystofthepancreas）肿瘤（Tumors）103Normalcommonbileductandpancreaticduct104急性水肿性（间质性）胰腺炎

acuteedemouspancreatitis暴饮暴食（酗酒）；胆道结石间质充血水肿炎细胞浸润（中性粒细胞和单核细胞）可局灶性脂肪坏死预后好105Acutehemorrhagicpancreatitis106Hemorrhageandnecrosis107Lipasereleasedfromthepancreaticacinicausesthedevelopmentoffocioffatnecrosis.Thisisseenaswhitespots.108109Histologicallythesespotsarecomposedofnecroticadiposetissue,withadjacentreactiveinflammation.110特点：发病急，病情重病理：胰腺肿大，结构模糊；