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••Biliary diseasesBiliary diseases

Liver Pathology 4:Liver Pathology 4:yy

••Vascular diseasesVascular diseases••ParasitesParasites••TumorsTumorsTumorsTumors••Liver transplantationLiver transplantation

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Causes of Large Bile Duct Obstruction

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Extrahepatic Biliary Atresia (EHBA)

•Reovirus 3•Rotavirus•Immune targeting?

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KasaiHepaticPortoenterostomy

Loop of smallintestine

Portoenterostomy

EHBARx Liver Transplantation

Primary Sclerosing Cholangitis (PSC)

ERCP: endoscopic retrogradecholangiopancreatography

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, fewer Crohn’s

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Primary Biliary Cirrhosis(PBC)

Guys’ Hospital ReportLondon, 1851

Addison & Gull

Mrs. Elizabeth Peacock

PBC: Primary biliary cirrhosis----Chronic nonsuppurative destructive cholangitis

•middle-aged women+ AMA (anti-mitochondrial

antibodies); M2 subtype

PDC-E2MolecularMimicry?

); yp• Alkaline Phosphatase• IgM•AMA directed against innermitochondrial membranePDC-E2 (pyruvate dehydrogenasecomplex-E2)

HLAII

•Assoc. with other autoimmune

Gram –bacteria

florid bileduct lesion

bile duct

AMAT TT

ssoc. w t ot e auto u edis: e.g. RA, sicca, celiac, scleroderma

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Vascular Disease &the Liver

•Veins•Veins•Sinusoids•Arteries

•Liver is often involved in heart disease or other outflow problems(“hepatic venousoutflow obstruction”)

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Budd-Chiari Syndrome(Hepatic venous outflowobstruction)

•oral contraceptives•coagulopathy (prot. S, C,

factor V Leiden def.,anti-cardiolipin Ab’s

•tumor invasion of hep. V’s

ascitesor IVC: renal cell CA + HCC

•small vein disease: VOD

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Peliosis hepatis: blood lakes of the liver-HIV, C17-alkylated steroids (OC’s,

anabolic steroids; systemic inflamm.

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Infarct: Arterial disease (PAN, ligation,chemoRx installation)Portal vein: Zahn infarct

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P.V.P.V.

Pylephlebitis:-perforated ulcer, -ruptured appendix, etc.

Pylephlebitis:-perforated ulcer, -ruptured appendix, etc.

S i i i i iSchistosomiasis: Pre-sinusoidal,intrahepaticportal hypertension

(Symmers’ “clay pipestem fibrosis”)

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pancreas

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LIVER-CELL ADENOMA-oral contraceptives-hepatocyte nuclear factor 1α (HNF-1α)

mutations-benign hepatocytes bld vessels NO b d ’s-benign hepatocytes, bld vessels, NO b.d. s

FOCAL NODULAR HYPERPLASIA (FNH)-malformation: central scar with artery,

outgrowth of cirrhosis-like mass

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HCC

•75% of pts have cirrhosis•75% are serum AFP +•Risks: Cirrhosis due to HBV, HCV alcohol hemochromatosisHCV, alcohol, hemochromatosis•Invades veins: portal vein/hepatic vein/IVC- to lungs

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Hepatocellular carcinoma (HCC)

FIBROLAMELLAR Ca: young, no risks-better prognosis with resection/tx

Nocirrhosis

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Cholangiocarcinoma(bile duct Ca)

Cholangiocarcinomaassociated with:•Liver flukes:

-Clonorchis sinensis-Opisthorcis viverrini

•PSCGallstone disease•Gallstone disease,hepatolithiasis

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Liver Transplantation

Ci h i•Cirrhosis-HBV, HCV

• PBC• PSC• Extrahepatic biliary

atresiaatresia• AFLD/NAFLD•Metabolic disorders

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Acute Rejection

endothelium

Portal tract

HLA II

TBD

endothelium

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1. Acute rejection: triadinflammation

bile duct damage

Liver Transplantation PathologyLiver Transplantation Pathology

1. Acute rejection: triad2. Preservation injury

(ischemia/reperfusion)3. CMV hepatitis

6 mos.

b e duct da ageendotheliitis

4. Recurrent original disease5. De novo autoimmune hepatitis6. Chronic rejection

DameSheila SherlockSheila Sherlock

The Royal FreeHospitalLondon, U.K.

“Diseases of the Liver & Biliary System”

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Peter J. Scheuer Hans Popper

Peter J. Scheuer, M.D.

Royal Free HospitalProf. of Histopathology

“Liver Biopsy Interpretation”-1st Edition: 1968-7th Edition: 2005

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