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Normal structure of liver Normal function of liver Metabolic
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tarix | 03.11.2018 | ölçüsü | 5,24 Mb. | | #77903 |
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Normal function of liver Metabolic (glucose homeostasis…) Synthetic (albumin, coagulation factors) Storage (glycogen, triglycerides, Fe, Cu, vitamins soluble in fat) Catabolic (endogenous substances – hormones…, exogenous substances – drugs, alcohol…) Excretoric (bile)
Histologic examination Necropsy Excision during laparotomy Fine needle punction Percutaneous punction biopsy unguided laparoscopic guided (Sono, CT) transjugular
Representativeness Biopsy represents approx. 1/500 000 of organ Ideal biopsy (Scheuer, MacSween) 2 cm length more than 4 (8) portal tracts Borderline representativeness contact CLINICIAN!! Non-representative
Quality Clinical data Clear and sufficient clinical outcome, history etiology biochemical findings, other markers (oncomarkers, markers of autoimmunity…) treatment previous histology preliminary diagnosis! Unclear contact CLINICIAN!! Insufficient and confusing
Quality Diagnosis Diagnosis recent diagnostic praxis (grading and staging of chronic hepatitis…) recent diagnostic textbooks - contact CLINICIAN!! Methods biopsy - contact CLINICIAN!! immunohistochemistry, ELMI - contact CLINICIAN!! experimental
Recently used diagnostic textbooks
Histologic examination – special stains and methods Haematoxylin/eosin Fouchet – bile (green), collagen (red) Fe, Cu – Fe pigment, Cu pigment Gömori – reticulin mesh Orcein – HBsAg PAS, Müller – mucopolysaccharides Immunohistochemistry (HBsAg, HBcAg, 1 fetoprotein, CEA, hepatocytes – OCH1E5) ELMI (metabolic diseases) PCR (HBV DNA, HCV RNA,…)
Damage of hepatocytes Type (ischemic-coagulation necrosis, piecemal necrosis, apoptosis) Extent (monocellular, focal, submassive, massive, bridging: C-C; C-P) Localization (centrolobular, periportal, midzonal) Course regeneration, healing ad integrum regeneration + fibrosis: cirrhosis acute liver failure: coma and death
Liver failure Jaundice Hypoalbuminemia Coagulopathy Hyperamonemia Increase of levels of cytosolic enzymes Endocrine complications Hepatoencephalopathy Hepatorenal syndrome Coma
Necrosis of liver: damage of hepatocytes
Cirrhosis Nodular rearrangement Morphology micronodular macronodular
Cirrhosis: etiology Alcohol (60-70%) Chronic hepatitis (10%) Cryptogenic (10-15%) Biliary (primary, secondary) Haemochromatosis (primary, secondary) Metabolic diseases (tyrosinemia, galactosemia) -very rare
Cirrhosis: complications Portal hypertension Liver failure Hepatocellular carcinoma
Portal hypertension Prehepatic (tumor, infection, pancreatitis, pregnancy, peroral contraceptives) Intrahepatic – most frequent (cirrhosis, fibrosis) Posthepatic (congestive cardiac failure, Budd-Chiari syndrome, venoocclusive disease) Signs: portosystemic shunts splenomegaly ascites
Cirrhosis
Cirrhosis
Cirrhosis
Cirrhosis + hepatocellular carcinoma
Cirrhosis: Masson staining
Cirrhosis: Fouchet staining
Cirrhosis
Cirrhosis: active
Cirrhosis: biliary
Dysplasia
Dysplasia
Hepatitis: etiology Infectious (acute, chronic) viruses (most often) bacteria (TBC, malaria, salmonelosis, leptospirosis,…) parazites (ecchinococcus) protozoal (amebiasis) Non-infectious (acute, chronic) autoimmune metabolic drug induced cryptogenic
Viral hepatitis
Viral hepatitis: HBV+HDV
Viral hepatitis: HBsAg, Orcein staining
Viral hepatitis: HBcAg, immunohistochemistry
Viral hepatitis: HBcAg, electron microscopy
Viral hepatitis: course Acute (HAV, HBV, HCV, HDV, HEV) Chronic (HBV, HBV + HDV, HCV) risk of cirrhosis development risk of hepatocellular carcinoma development Fulminant (HBV, HCV)
Acute hepatitis Gross finding – oedematous, red liver Microscopic finding focal necrosis bridging necrosis panacinar/multiacinar necrosis periportal necrosis Hepatocytes polymorphism balooning degeneration
Acute hepatitis Inflammation polynuclear/mononuclear infiltration activation of Kupfer’s cells Other changes cholestasis damage of bile ducts Regeneration
Fulminant hepatitis Gross finding - soft consistency Microscopic finding – complete necrosis of parenchyma Course liver failure - coma - death regeneration – postnecrotic cirrhosis
Acute hepatitis: HBV
Acute hepatitis
Acute hepatitis
Acute hepatitis
Fulminant hepatitis: HBV
Fulminant hepatitis: HBsAg, Orcein staining
Chronic hepatitis: etiology HBV, HBV+HDV, HCV Autoimmune Drug induced Wilson’s disease Cryptogenic
Chronic hepatitis: clinical definition Clinical symptoms of hepatitis more than: 6 months 12 months in HCV
Chronic hepatitis: morphology Inflammation (see transparency) portal periportal lobular Necrosis/apoptosis piecemal bridging focal Fibrosis (see transparency) Deposits (Cu, PAS globules,…)
Chronic hepatitis: classification (see transparency)
Chronic hepatitis - portal + steatosis, HCV
Chronic hepatitis: portal
Chronic hepatitis: periportal
Chronic hepatitis: periportal
Chronic hepatitis: HCV, transition into the cirrhosis
Autoimmune chronic hepatitis Females, young/middle age Autoantibodies – ANA, AMA Autoimmune „overlap“ syndromes lupus erythematodes arthritis
Autoimmune chronic hepatitis - ANA
Autoimmune chronic hepatitis - AMA
NASH: non-alcoholic steatohepatitis Hepatomegaly Increase of aminotransferases Histological picture – similar to alcoholic hepatitis without alcohol consumption macrovesicular steatosis lobular hepatitis and necrosis balooning of hepatocytes fibrosis
NASH: non-alcoholic steatohepatitis Epidemiologic signs of NASH Females Obesity
NASH: non-alcoholic steatohepatitis Diseases related to NASH Acquired metabolic diseases (obesity, DM, hyperlipidemia, parenteral nutrition) Inherited metabolic diseases (Wilson’s disease, abetalipoproteinaemia, tyrosinaemia) Surgery (jejunoileal bypass, extensive resections of small intestine...) Drugs, toxins (amiodarone, glucocorticoids, tamoxifen, synthetic estrogens…)
Toxic and drug induced liver damage Anorganic substances Organic substances Industrial and plant substances Poisons/venoms Drugs Alcohol
Toxic and drug induced liver damage: effect Expectable Non-expectable (idiosyncratic)
Toxic and drug induced liver damage : forms Necrosis Steatosis macrovesicular (alcohol, corticoids) microvesicular (tetracyklin, aspirine) Cholestasis (steroids-anabolic, contraceptives) Hepatitis mild (aspirin, synthetic penicilines) Hepatitis serious (halotan) Chronic periportal hepatitis (sulphonamides)
Toxic and drug induced liver damage : forms Granulomatous hepatitis (phenylbutazone) Vascular lesions (contraceptives, cytostatics) Hyperplasia, neoplasia (contraceptives)
Alcoholic Liver Disease (ALD) Steatosis (90%) Fibrosis (perivenular, pericellular) Steatofibrosis Alcoholic hepatitis Alcoholic cirrhosis Mallory hyaline Cholestasis Siderosis Hepatocellular carcinoma Dif.dg: NASH
Alcoholic Liver Disease (ALD) : steatosis
Alcoholic Liver Disease (ALD) : steatosis
Alcoholic Liver Disease (ALD) : steatofibrosis
Alcoholic Liver Disease (ALD) : cholestasis
Alcoholic Liver Disease (ALD) : Mallory hyaline
Alcoholic Liver Disease (ALD) : Mallory hyaline
Alcoholic Liver Disease (ALD) : steatohepatitis
Alcoholic Liver Disease (ALD) : steatohepatitis
Alcoholic Liver Disease (ALD) : steatohepatitis, cholestasis, Mallory hyaline
Metabolic liver disease Haemochromatosis Wilson’s disease 1 anti-trypsin deficiency Glycogenoses
Haemochromatosis Excessive accumulation of iron in parenchymatous organs Primary (idiopatic), secondary Cirrhosis, diabetes mellitus, pigmentation of skin Dif.dg. of brown pigment within the liver Fe (haemochromatosis) Cu (Wilson’s disease) lipopigment (brown atrophy of liver, lipopigment dystrophy)
Haemochromatosis
Haemochromatosis
Haemochromatosis
Brown atrophy of liver: dif.dg.
Wilson’s disease Hepatolenticular degeneration Low levels of ceruloplasmin Increase of Cu in liver and urine Morphology steatosis acute hepatitis chronic hepatitis glycogen nucleus (also typical in DM) increased amount of Cu pigment
Wilson’s disease : Cu pigment
Wilson’s disease/DM: glycogen nucleus
Wilson’s disease/DM: glycogen nucleus
1 anti-trypsin deficiency Low levels of 1AT (inhibitor of proteases) Emphysema of lungs Liver changes (cholestasis, cirrhosis) PAS positive acidophilic globular inclusions in the cytoplasm ELMI – complementary diagnosis
Circulatory disorders of the liver Disorders of arterial system Disorders of portal system Disorders of venous system Disorders of lobular system
Disorders of arterial system Infarct of the liver Rare Thrombosis, compression, obliteration of a. hepatis branch (polyarteritis nodosa,…) Pale Dif.dg.: Zahn infarct (occlusion of intrahepatic branch of v. portae): no necrosis, local congestion of sinusoids and secondary atrophy
Liver infarct
Disorders of portal system Thrombosis and occlusion of v.portae Extrahepatal (phlebitis, pancreatitis, surgery, trauma) Intrahepatal (invasion of tumor)
Thrombosis of v.portae
Disorders of venous system Thrombosis of hepatic veins (Budd-Chiari syndrome) causes: polycytemia vera, pregnancy, contraceptives, intraabdominal malignant tumors, hepatocellular carcinoma centrilobular necrosis and congestion, fibrosis Venoocclusive disorder complication of bone marrow transplantation (5-25%) – chemotherapy, radiotherapy Bush-tea (pyrrolizidine alcaloids) obliteration of hepatic veins – subendothelial accumulation of colagen and reticulin, perivenular fibrosis, perivenular hemosiderin
Budd-Chiari
Disorders of lobular system Occlusion of sinuses eclampsy Sickle cell anaemia Systemic circulation passive congestion centrilobular necrosis nutmeg liver (venostasis/steatosis)
Congestion
Pathology of intrahepatal bile ducts Secondary biliary cirrhosis (SBC) Primary biliary cirrhosis (PBC) Primary sclerosing cholangitis (PSC)
SBC Obstruction of extrahepatal bile ducts: atresia, lithiasis, tumor, iatrogenic Striking cholestasis
PBC Autoimmune (AMA), Coincidency: Sjögren sy., arthritis, thyreoiditis, vasculitis,.. Hepatomegaly, 4 stages I - granulomatous destruction of ducts II - ductular proliferation and periportal hepatitis III - scarring, necrosis, septal fibrosis IV - cirrhosis
PSC Autoimmune (ANA) Inflammation and obliterative fibrosis of bile ducts Coincidency: IBD (Inflammatory Bowel Disease – ulcerative colitis, m.Crohn)
PBC: I.stage
PBC: I.stage
PBC: II.stage
PBC: III.stage
Patology of liver during pregnancy Preeclampsy/eclampsy HELLP syndrome: Hemolysis, Elevated Liver enzymes, Low Platelets fibrin within the sinusoids hematomas (fatal rupture of liver) Acute microvesicular steatosis during pregnacy (third trimester) Cholestasis during pregnancy (third trimester)
Transplantation Liver disorders related to bone marrow transplantation Liver disorders related to liver transplantation
Liver disorders related to bone marrow transplantation
Liver disorders related to liver transplantation
Tumors of liver Tumor-like lesion Benign tumors Malignant tumor
Tumor-like lesions Focal nodular hyperplasia Nodular regeneratory hyperplasia (lack of fibrosis) Cysts Hamartoma (von Meyenburg complex) Inflammatory pseudotumor
Nodular regeneratory hyperplasia
Benign tumors Adenoma hepatocellular (lack of portal tracts!) cholangiogenic (lack of bile production, less than 1cm, subcapsular) Haemangioma subcapsular cavernous bleeding risk during punction!
Cavernous haemangioma
Malignant tumors Hepatocellular carcinoma (HCC) Cholangiogenic carcinoma (lack of bile production) peripherial extrahepatal -Klatskin tumor Hepatoblastoma (embryonal, teratoid,…) Angiosarcoma, malignant haemangioendothelioma Metastases (most often) GIT, lung, kidney
HCC
HCC
HCC
HCC
Cholangiogenic carcinoma
Biliary system: cholelithiasis 10-20% of population Cholesterol concrements (females, obesity, steroids, hyperlipidemia) Pigmented/bilirubine concrements (biliary infection, haemolysis) Blockage of bile ducts (acute cholangoitis, sepsis, biliary cirrhosis, pancreatitis) Irritation of gallbladder (cholecystitis, carcinoma) Valve-like blockage (hydrops) Perforation, fistula
Cholecystolithiasis
Cholecystolithiasis
Cholecystitis Acute empyema of gallbladder gangrenous cholecystitis acalculous cholecystitis (postoperative, trauma, burns, sepsis,…) Chronic fibroproduction (thickening of the wall, adhesion) chronic inflammmation dystrophic calcification (risk of carcinoma development) hydrops
Chronic cholecystitis
Carcinoma of gallbladder Adenocarcinoma Infiltrating Exophytic Early invasion into the liver – poor prognosis!
Carcinoma of gallbladder
Other disorders of biliary system Atresia of bile ducts (rapid progression, uncelar etiopathogenesis, cirrhosis) Cholesterolosis (strawberry gallbladder) Choledocholithiasis (risk of ascendent cholangoitis, intrahepatic abscess and sepsis, risk of biliary cirrhosis) Tumors of papilla Vateri (ampuloma, periampular carcinoma – difficult diagnosis)
Exocrine pancreas: acute pancreatitis Causes cholelithiasis, alcohol, surgery, trauma, idiopathic Most serious form acute haemorrhagic necrotizing pancreatitis Morphology nekrosis of pancreas nekrosis of fat tissue (steatocytonecrosis, Balzer’s necrosis) haemorrhagy Complication, healing abscesses pseudocysts multiorgan failure
Pancreatonecrosis
Pancreatonecrosis
Steatocytonecrosis: omentum
Steatocytonecrosis: omentum
Exocrine pancreas: chronic pancreatitis Causes alcohol, hyperlipidemia, hypercalcemia, hereditary Morphology fibrosis reduction of acines obstruction of ducts chronic inflammatory infiltration (lymphocytes, plasmocytes) Complications calcifications, pseudocysts, thrombosis v . lienalis diabetes steatorrhea jaundice
Chronic pancreatitis
Exocrine pancreas: tumors Cystic tumors (5%) mucinous cystadenoma mucinous cystadenocarcinoma Carcinoma of pancreas Adenocarcinoma, various degree of differentiation Head (early diagnosis - jaundice), body, tail (difficult diagnosis, late detection) Often - fibroproduction (dif.dg. chron.pancreatitis) Trosseau syndrome – migratory thrombophlebitis in 10%
Carcinoma of pancreas
Carcinoma of pancreas
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