肝脏病理-ppt.ppt

Welcome肝脏疾病病理学诊断Outline of GuidelinesClassification of liver diseases LIVERLiver BiopsyLiver Biopsy Confirm the diagnosis Serological blood tests Establish degree of fibrosis Serology Biopsy not representative Identify cirrhosis Determine optimum therapyLiver biopsy adequacy“Most hepatopathologists are satisfied with a biopsy specimen containing at least six to eight portal tracts”.Bravo AA et al NEJM 344,495;2001.We should not be primarily concerned with the size of biopsy that“satisfies”the pathologist.The correct question is:What size of biopsy will provide a reliable assessment for the patients management?Liver biopsy adequacyGuido M and Rugge M.Semin Liv Dis 24,89;2004.In most diffuse liver diseases examination of 12-15 complete portal tracts is necessary.20mm of a 1.4mm diameter(17 gauge)needle biopsy.Progressively longer samples of thinner biopsies are needed.36-1 肝脏活检标本的评价整体情况-低倍镜汇管区 中、高倍镜肝小叶 中、高倍镜中央静脉 中、高倍镜LOBULE VS ACINUS肝小叶肝板、肝窦肝脏,EM肝脏,EM(cont.)汇管区和中央静脉36-2 常用诊断名词的定义 嗜酸性小体气球样变性Ductule叶间胆管碎宵状坏死n灶状坏死n带状坏死n融合性坏死n大片坏死n桥接坏死 Acidophil bodyBallooning DegenerationFatty Degeneration Alcoholic liver diseaseDuctuleInterlobular bile ductNecrosis,piecemealNecrosis,focalNecrosis,ZonalNecrosis,ConfluentNecrosis,MassiveNecrosis,BridgingDrug and Toxin-Induced Liver Disease36-3 肝小叶淋巴细胞浸润伴有或不伴有肝细胞变性或坏死急性医源性急性病毒性(HAV,HBVB,HCV,CMV,EBV etc)自身免疫性癌髓外造血白血病/淋巴瘤原发性胆管性肝硬化36-4 肝小叶多形核细胞浸润伴有或不伴有肝细胞变性或坏死酒精性肝炎细菌/真菌感染医源性反应败血症“手术性”肝炎病毒感染（CMV)Autoimmune Hepatitis 36-5 肝细胞坏死伴轻微炎症反应急性病毒感染纤维化性胆汁淤积性肝炎肝静脉血液外溢缺血嗜肝病毒引起的大片坏死医源或毒素反应创伤肿瘤36-6 汇管区淋巴细胞或和浆细胞浸润急性病毒性肝炎自身免疫性肝炎胆管阻塞移植物抗宿主反应淋巴瘤/白血病肝小叶炎症或变性或坏死轻微n肉芽肿或肿瘤n原发性胆汁性肝硬化n原发性硬化性胆管炎n排斥反应n病毒性肝炎nWilson病Primary biliary cirrhosis Antimitochondrial antibody36-7 汇管区多形核细胞浸润-肝小叶炎症或变性或坏死轻微顺行性胆管炎胆道阻塞高营养医源性病毒性肝炎,“胆管溶解性”36-8 汇管区嗜酸性粒细胞浸润自身免疫性肝炎髓外造血医源反应寄生虫感染原发性胆汁性肝硬化原发性硬化性胆管炎排斥反应Liver biopsy from a 25 y-o Laotian showing a granulomatous reaction against the ovum(HE).36-9 肉芽肿性炎症儿童慢性肉芽肿病克隆氏病异物反应原发性肝脏肉芽肿病免疫性胆管炎细菌、真菌、立克次氏体、病毒感染脂肪性肉芽肿恶性肿瘤医源性反应原发性胆汁性肝硬化肉瘤样病36-10 肝纤维化淀粉样桥接性纤维化中央透明变性纤维化先天性肝纤维化先天性梅毒囊性纤维化灶性结节状增生肝脏门脉区纤维化肝脏静脉性血液外溢，慢性阻塞性代谢性疾病中央静脉周围纤维化肿瘤肝纤维化肝硬化=肝细胞再生+纤维化36-11 胆汁淤积良性家族性胆汁淤积妊娠性胆汁淤积医源性手术后性胆汁淤积败血症细胞内淤胆Intrahepaptic CholestasisExtrahepaptic Cholestasis36-12 淤血或出血伴有肝窦扩张肝静脉血外溢静脉阻塞性疾病心力衰竭医源反应肿瘤压迫结节性再生性增生和回管区硬化门脉阻塞肝淤血肝出血36-13 色素沉积胆色素铁脂褐素其它外源性色素福尔马林色素胆色素 bile pigmentHemochromatosisPrussian blue iron stain Lipofuscin36-14 细胞内包含体腺病毒抗胰靡蛋白酶缺乏支链淀粉血症大泡脂肪变微泡脂肪变“糖原性”胞核巨线粒体“毛玻璃”细胞HSVMallory hyalineALPHA-1-ANTITRYPSIN STORAGE MEGAMITOCHONDRIA GROUND GLASS CELLS Alpha-1-antitrypsin deficiencyCMV hepatitis Glycogen NucleiA 30-year-old woman in the 30th week of pregnancy with Fulminant hepatic failure herpes simplex virusMallory hyaline36-15 脂肪变显著不伴有或轻微坏死酒精性脂肪性肝炎妊娠脂肪肝灶性脂肪变肝细胞腺瘤、癌医源、毒素反应代谢性疾病非酒精性脂肪性肝炎非特异性脂肪变Wilson病36-16 组织中出现不常见细胞髓外造血巨核细胞转移瘤储备细胞（肝、Kupffer或Ito)髓外造血36-17“几乎正常”的肝活检肝汇管区纤维化医源性反应Missed lesion结节状再生或增生蓄积或代谢性疾病36-18 缺少(观察不到)正常结构胆管中央静脉肝细胞汇管区汇管静脉肝窦36-19 肝脏代谢性疾病的光镜诊断类型类型诊断性诊断性LM诊断性诊断性EMa-抗胰糜蛋白酶缺乏抗胰糜蛋白酶缺乏汇管区周肝细胞内汇管区周肝细胞内PAS阳性球滴阳性球滴扩张内质网内出现颗粒状物扩张内质网内出现颗粒状物囊性纤维化囊性纤维化灶性胆管性纤维化灶性胆管性纤维化胆管纤维状物胆管纤维状物Gaucher病病Kupffer增大，汇管区巨噬细胞，增大，汇管区巨噬细胞，“Crinkled paper”胞浆胞浆溶酶体内管状包含体溶酶体内管状包含体糖原沉积症糖原沉积症 IV嗜碱性细胞内包含物嗜碱性细胞内包含物,PSA+丝状物丝状物粘多糖贮积症粘多糖贮积症肝细胞肝细胞,Kuppfer 胶体铁染色胶体铁染色溶酶体内溶酶体内“羊毛羊毛”状物状物迟发性皮肤卟啉症迟发性皮肤卟啉症肝细胞内针状包含物肝细胞内针状包含物肝细胞内针状包含物肝细胞内针状包含物红细胞生成性原卟啉症红细胞生成性原卟啉症胆素色胆素色(毛细核小胆管毛细核小胆管)“Starburst”状排列的结晶状排列的结晶36-20 肝脏代谢性疾病的电镜和非特异性光镜诊断类型类型LMEM糖原沉积糖原沉积,II细胞内糖原增加细胞内糖原增加结合溶酶体结合溶酶体,糖原糖原GM2神经节苷脂沉积症神经节苷脂沉积症正常正常膜性层状包含体膜性层状包含体Niemann-Pick 病病泡沫状肝、泡沫状肝、Kupffer细胞细胞溶酶体内溶酶体内“myelin”样包含体样包含体Wolman病病泡沫状肝、泡沫状肝、Kupffer细胞细胞肝、肝、Kupffer细胞内脂滴细胞内脂滴Zellweger综合症综合症非特异性肝细胞改变非特异性肝细胞改变缺乏过氧化小体缺乏过氧化小体(peroxisomes)36-21 肝脏代谢性疾病非诊断的光镜和电镜特点类型LMEM半乳糖血症Galactosemia同高酪氨酸血症同高酪氨酸血症糖原沉积(I,III)Glycogenstorage肝细胞大,窦受压,铺转排列,脂肪变,高糖原核仁糖原分隔细胞器,脂滴,高糖原核仁遗传性果糖不耐症Hereditary fructose intolerance同高酪氨酸血症“fructoseholes”同心排列内质网,糖原颗粒高酪氨酸血症Tyrosinemia肝细胞变性伴脂肪变(灶性),淤胆,纤维化,肝硬化淤胆,脂滴,内质网增加,异常线粒体高酪氨酸血症肝脏Glycogen storage,Liver,LMGlycogen storage,Liver,EM肝脏疾病诊断病因学形态（LM&EM)组化和免疫组化血清学、酶学检查临床表现Alcoholic Fatty LiverAlcoholic Fatty LiverCirrhosisFibrosisRegeneratingNoduleMicronodular cirrhosisMicronodular cirrhosis:Hepatic AdenomaHepatocellular CarcinomaHepatic metastasis:Hepatic metastasis:Amoebic Liver Abscess:Hepar Lobatum(cong.Syphilis):HCC-The Global Perspective The Big Five CancersThe Major Etiological FactorsChronic hepatitis -types B or CCirrhosis/chronic liver disease of any type Aflatoxin exposureMales,increasing age8%-High 2-7%-Intermediate 2%-LowData from CDC Chronic HBV Infection-Geographic DistributionProspective Study of HCC Development in HBsAg Seropositive Male Chinese HCC DEVELOPMENT 19,223(HBsAg-ve)9 22,707 Mean follow-up=8.9 years Male Chinese 3,454(HBsAg+ve)152 Relative risk=98.4(50.2-193)Beasley,1982Beasley,1982Beasley 1986“The lifetime risk of developing Hepatocellular carcinomaIn a Chinese male carrier Of the hepatitis B virusIs between 40 and 50%”Chronic Viral Hepatitis&HCCOkuda,1999Anti-HCV Prevalence5%-High 1.1-5%-Intermediate 0.2-1%-Low 0.2-1%-LowChronic HCV Infection-Geographic Distribution Thank Your Attendance!肝脏肿瘤-Neoplasms of the Liver Hepatocellular Carcinoma and CholangiocarcinomaPathology of Vascular Diseases of the Liver Referenceshttp:/