Liver Pathology 4:Liver Pathology 4:••Biliary diseasesBiliary diseases
gygy••Biliary diseasesBiliary diseases••Vascular diseasesVascular diseasesVascular diseasesVascular diseases••ParasitesParasites••TumorsTumors••Liver transplantationLiver transplantation
Causes of Large Bile Duct Obstruction
Extrahepatic Biliary Atresia (EHBA)Biliary Atresia (EHBA)
•Reovirus 3•RotavirusI•Immune targeting?
Loop of smallintestine
KasaiH tiHepaticPortoenterostomy
EHBARx Liver TransplantationRx
Primary Sclerosing Cholangitis (PSC)
ERCP: endoscopic retrogradecholangiopancreatography
, fewer Crohn’s
Primary Biliary CirrhosisPrimary Biliary Cirrhosis(PBC)
Guys’ Hospital ReportLondon, 1851
Addison & GullAddison & Gull
Mrs. Elizabeth Peacock
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
Vascular Disease &the Liver
•Veins•Sinusoids•Arteries
•Liver is often involved in heart disease or other outflow problems(“hepatic venous( poutflow obstruction”)
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
Peliosis hepatis: blood lakes of the liver-HIV, C17-alkylated steroids (OC’s,HIV, C17 alkylated steroids (OC s,
anabolic steroids; systemic inflamm.
Infarct: Arterial disease (PAN, ligation,h R i t ll ti )chemoRx installation)
Portal vein: Zahn infarct
P.V.P.V.
Pylephlebitis:Pylephlebitis:Pylephlebitis:-perforated ulcer, ruptured appendix etc
Pylephlebitis:-perforated ulcer, ruptured appendix etc-ruptured appendix, etc.-ruptured appendix, etc.
Schistosomiasis: Pre-sinusoidal,,intrahepaticportal hypertensionportal hypertension
(Symmers’ “clay pipestem fibrosis”)
pancreas
LIVER-CELL ADENOMA-oral contraceptivesp-hepatocyte nuclear factor 1α (HNF-1α)
mutations-benign hepatocytes, bld vessels, NO b.d.’s
FOCAL NODULAR HYPERPLASIA (FNH)malformation: central scar with artery-malformation: central scar with artery,outgrowth of cirrhosis-like mass
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
Hepatocellular carcinoma (HCC)
FIBROLAMELLAR Ca: young, no risks-better prognosis with resection/txbetter prognosis with resection/tx
NNocirrhosis
CholangiocarcinomaCholangiocarcinoma(bile duct Ca)
Cholangiocarcinomai t d ithassociated with:
•Liver flukes:-Clonorchis sinensis
-Opisthorcis viverrini
•PSC•Gallstone disease,hepatolithiasis
Liver Transplantation
•Cirrhosis-HBV, HCVHBV, HCV
• PBC• PSC• Extrahepatic biliary
atresiaA / A• AFLD/NAFLD
•Metabolic disorders
Acute Rejection
HLA II endothelium
TBD
Portal tractT
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
DameSheila Sherlock
The Royal FreeHospitalpLondon, U.K.
“Diseases of the Liver & Biliary System”
Peter J. Scheuer Hans Popper
Peter J. Scheuer, M.D.
Royal Free HospitalProf. of Histopathology
“Liver Biopsy Interpretation”“Liver Biopsy Interpretation”-1st Edition: 1968-7th Edition: 20057 Edition: 2005