Normal structure of liver Normal function of liver Metabolic



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Normal structure of liver


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, ground glass



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



Acute hepatitis: regeneration



Fulminant hepatitis: HBV



Fulminant hepatitis: HBsAg, Orcein staining



Chronic hepatitis: etiology



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

  • DM of II type, hyperglycaemia



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),

  • Majority of females

  • 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)

  • subvariant – fibrolamelar carcinoma

  • 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

  • duodenal obstruction

  • 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



  • http://ustavpatologie.upol.cz



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