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Liver Pathology 4:Liver Pathology 4:••Biliary diseasesBiliary diseases
gygy••Biliary diseasesBiliary diseases••Vascular diseasesVascular diseasesVascular diseasesVascular diseases••ParasitesParasites••TumorsTumors••Liver transplantationLiver transplantation
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Causes of Large Bile Duct Obstruction
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Extrahepatic Biliary Atresia (EHBA)Biliary Atresia (EHBA)
•Reovirus 3•RotavirusI•Immune targeting?
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Loop of smallintestine
KasaiH tiHepaticPortoenterostomy
EHBARx Liver TransplantationRx
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Primary Sclerosing Cholangitis (PSC)
ERCP: endoscopic retrogradecholangiopancreatography
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Primary Biliary CirrhosisPrimary Biliary Cirrhosis(PBC)
Guys’ Hospital ReportLondon, 1851
Addison & GullAddison & Gull
Mrs. Elizabeth Peacock
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PBC: Primary biliary cirrhosis----Chronic nonsuppurative destructive cholangitis
•middle-aged women+ AMA (anti-mitochondrial
PDC-E2MolecularMimicry?
antibodies); M2 subtype• Alkaline Phosphatase• IgM
Gram –bacteriaIgM
•AMA directed against innermitochondrial membranePDC E2 ( t d h d HLAPDC-E2 (pyruvate dehydrogenasecomplex-E2)
bile duct
HLAII
•Assoc. with other autoimmunedis: e.g. RA, sicca, celiac, scleroderma
T
g , , ,
florid bileduct lesion
AMAT TT
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Vascular Disease &the Liver
•Veins•Sinusoids•Arteries
•Liver is often involved in heart disease or other outflow problems(“hepatic venous( poutflow obstruction”)
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Budd-Chiari Syndrome(H ti tfl(Hepatic venous outflowobstruction)
•oral contraceptives( S C•coagulopathy (prot. S, C,
factor V Leiden def.,anti cardiolipin Ab’santi-cardiolipin Ab s
•tumor invasion of hep. V’sor IVC: renal cell CA + HCC
ascites•small vein disease: VOD
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Peliosis hepatis: blood lakes of the liver-HIV, C17-alkylated steroids (OC’s,HIV, C17 alkylated steroids (OC s,
anabolic steroids; systemic inflamm.
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Infarct: Arterial disease (PAN, ligation,h R i t ll ti )chemoRx installation)
Portal vein: Zahn infarct
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P.V.P.V.
Pylephlebitis:Pylephlebitis:Pylephlebitis:-perforated ulcer, ruptured appendix etc
Pylephlebitis:-perforated ulcer, ruptured appendix etc-ruptured appendix, etc.-ruptured appendix, etc.
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Schistosomiasis: Pre-sinusoidal,,intrahepaticportal hypertensionportal hypertension
(Symmers’ “clay pipestem fibrosis”)
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LIVER-CELL ADENOMA-oral contraceptivesp-hepatocyte nuclear factor 1α (HNF-1α)
mutations-benign hepatocytes, bld vessels, NO b.d.’s
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FOCAL NODULAR HYPERPLASIA (FNH)malformation: central scar with artery-malformation: central scar with artery,outgrowth of cirrhosis-like mass
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HCCHCC
•75% of pts have cirrhosis75% of pts have cirrhosis•75% are serum AFP +Ri k Ci h i d t HBV•Risks: Cirrhosis due to HBV,
HCV, alcohol, hemochromatosis•Invades veins: portal vein/hepatic vein/IVC to lungsvein/IVC- to lungs
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Hepatocellular carcinoma (HCC)
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FIBROLAMELLAR Ca: young, no risks-better prognosis with resection/txbetter prognosis with resection/tx
NNocirrhosis
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CholangiocarcinomaCholangiocarcinoma(bile duct Ca)
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Cholangiocarcinomai t d ithassociated with:
•Liver flukes:-Clonorchis sinensis
-Opisthorcis viverrini
•PSC•Gallstone disease,hepatolithiasis
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Liver Transplantation
•Cirrhosis-HBV, HCVHBV, HCV
• PBC• PSC• Extrahepatic biliary
atresiaA / A• AFLD/NAFLD
•Metabolic disorders
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Acute Rejection
HLA II endothelium
TBD
Portal tractT
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Liver Transplantation PathologyLiver Transplantation Pathology
inflammation
1. Acute rejection: triad2. Preservation injury
bile duct damageendotheliitisj y
(ischemia/reperfusion)3. CMV hepatitis3. CMV hepatitis
4 Recurrent original disease6 mos.
4. Recurrent original disease5. De novo autoimmune hepatitis6 Ch i j ti6. Chronic rejection
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DameSheila Sherlock
The Royal FreeHospitalpLondon, U.K.
“Diseases of the Liver & Biliary System”
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Peter J. Scheuer Hans Popper
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Peter J. Scheuer, M.D.
Royal Free HospitalProf. of Histopathology
“Liver Biopsy Interpretation”“Liver Biopsy Interpretation”-1st Edition: 1968-7th Edition: 20057 Edition: 2005