肝脏疾病病理学诊断.ppt

Titelmasterformat durch Klicken bearbeiten,Textmasterformate durch Klicken bearbeiten,Zweite Ebene,Dritte Ebene,Vierte Ebene,Fnfte Ebene,S.J.YANG 2006,Welcome,肝脏疾病病理学诊断,Outline of Guidelines,Classification of liver diseases,LIVER,Liver Biopsy,Liver Biopsy,Confirm the diagnosis,Serological blood tests,Establish degree of fibrosis,Serology,Biopsy not representative,Identify cirrhosis,Determine optimum therapy,Liver 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 patient,s management?,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,灶状坏死,带状坏死,融合性坏死,大片坏死,桥接坏死,Acidophil body,Ballooning Degeneration,Fatty Degeneration,Alcoholic liver disease,Ductule,Interlobular bile duct,Necrosis,piecemeal,Necrosis,focal,Necrosis,Zonal,Necrosis,Confluent,Necrosis,Massive,Necrosis,Bridging,Drug and Toxin-Induced Liver Disease,36-3 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,肉芽肿或肿瘤,原发性胆汁性肝硬化,原发性硬化性胆管炎,排斥反应,病毒性肝炎,Wilson,病,Primary biliary cirrhosis,Antimitochondrial antibody,36-7 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 Cholestasis,Extrahepaptic Cholestasis,36-12 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 pigment,Hemochromatosis,Prussian blue iron stain,Lipofuscin,36-14 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,HSV,Mallory hyaline,ALPHA-1-ANTITRYPSIN STORAGE,MEGAMITOCHONDRIA,GROUND GLASS CELLS,Alpha-1-antitrypsin deficiency,CMV hepatitis,Glycogen Nuclei,A 30-year-old woman in the 30th week of pregnancy with Fulminant hepatic failure,herpes simplex virus,Mallory hyaline,36-15 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,诊断性,LM,诊断性,EM,a-,抗胰糜蛋白酶缺乏,汇管区周肝细胞内,PAS,阳性球滴,扩张内质网内出现颗粒状物,囊性纤维化,灶性胆管性纤维化,胆管纤维状物,Gaucher,病,Kupffer,增大，汇管区巨噬细胞，“,Crinkled paper”,胞浆,溶酶体内管状包含体,糖原沉积症,IV,嗜碱性细胞内包含物,PSA+,丝状物,粘多糖贮积症,肝细胞,Kuppfer,胶体铁染色,溶酶体内“羊毛”状物,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,红细胞生成性原卟啉症,胆素色(毛细核小胆管),“Starburst”,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,LM,EM,糖原沉积,II,细胞内糖原增加,结合溶酶体,糖原,GM2,神经节苷脂沉积症,正常,膜性层状包含体,Niemann-Pick,病,泡沫状肝、,Kupffer,细胞,溶酶体内“,myelin”,样包含体,Wolman,病,泡沫状肝、,Kupffer,细胞,肝、,Kupffer,细胞内脂滴,Zellweger,综合症,非特异性肝细胞改变,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,LM,EM,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),Glycogen storage,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,“,fructose holes”,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,LM,Glycogen 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 Liver,Alcoholic Fatty Liver,Cirrhosis,Fibrosis,Regenerating Nodule,Micronodular cirrhosis,Micronodular cirrhosis:,Hepatic Adenoma,Hepatocellular Carcinoma,Hepatic metastasis:,Hepatic metastasis:,Amoebic Liver Abscess:,Hepar Lobatum(cong.Syphilis):,HCC-The Global Perspective,The Big Five Cancers,The Major Etiological Factors,Chronic hepatitis -types B or C,Cirrhosis/chronic liver disease of any type,Aflatoxin exposure,Males,increasing age,8%-High,2-7%-Intermediate,2%-Low,Data from CDC,Chronic HBV Infection,-,Geographic Distribution,Prospective 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,1982,Beasley 1986,“,The lifetime risk of developing,Hepatocellular carcinoma,In a Chinese male carrier,Of the hepatitis B virus,Is between 40 and 50%”,Chronic Viral Hepatitis&HCC,Okuda,1999,Anti-HCV Prevalence,5%-High,1.1-5%-Intermediate,0,.2-1%-Low,0.2-1%-Low,Chronic HCV Infection-Geographic Distribution,Thank Your Attendance!,肝脏肿瘤-,Neoplasms of the Liver,Hepatocellular Carcinoma and Cholangiocarcinoma,Pathology of Vascular Diseases of the Liver,References,