patoloji-ders-notlari

Title

Serdar Balcı

Circulatory and Cholestatic Disorders of Liver

Serdar BALCI, MD

CHOLESTATIC LIVER DISEASES

Cholestasis

Robbins and Cotran Pathologic Basis of Disease

Robbins Basic Pathology

Robbins and Cotran Pathologic Basis of Disease

Robbins and Cotran Pathologic Basis of Disease

Robbins Basic Pathology

Robbins Basic Pathology

Cholestatic Liver Diseases

Cholestatic hepatocytes are enlarged with dilated canalicular spaces.

Apoptotic cells.

Kupffer cells frequently contain regurgitated bile pigments

Robbins and Cotran Pathologic Basis of Disease

Intracellular cholestasis showing the bile pigments in the cytoplasm

Robbins and Cotran Pathologic Basis of Disease

Bile plug showing the expansion of bile canaliculus by bile

Robbins and Cotran Pathologic Basis of Disease

Autopsy Pathology: A Manual and Atlas

Large bile duct obstruction

Acute large duct obstruction. Marked edema, ductular reaction, neutrophils

Ascending cholangitis

Large bile duct obstruction → static bile → bacterial infections within the biliary tree

Primary hepatolithiasis

Disorder of intrahepatic gallstone formation

Most common in East Asia

Causes repeated bouts of ascending cholangitis and inflam­matory parenchymal destruction

Predisposes to cholangiocarcinoma

Neonatal Cholestasis

Neonatal hepatitis. Multinucleated giant hepatocytes

Cholestasis of Sepsis

Cholestasis of Sepsis Canalicular cholestasis

Bile plugs within predominantly centrilobular canaliculi

Prominent activated Kupffer cells

Mild portal inflammation

**Hepatocyte necrosis is scant or absent **

Robbins Basic Pathology

Cholestasis of Sepsis Ductular cholestasis

Canals of Hering and bile ductules at the interface of portal tracts and parenchyma become dilated and contain prominent bile plugs

Not a typical feature of biliary obstruction

Accompanies or even precedes the development of septic shock

Ductular cholestasis. Large, dark bile concretions within markedly dilated canals of Hering and ductules at the portal-parenchymal interface. This feature, indicative of current or impending severe sepsis, is related to endotoxemia.

Robbins Basic Pathology

AUTOIMMUNE CHOLANGIOPATHIES

Robbins Basic Pathology

PRIMARY BILIARY CIRRHOSIS (PBC)

Primary Biliary Cirrhosis (PBC)

Primary Biliary Cirrhosis

Biliary cirrhosis. Sagittal section through the liver demonstrates the nodularity and bile staining of end-stage biliary cirrhosis.

Robbins and Cotran Pathologic Basis of Disease

Unlike other forms of cirrhosis, nodules of liver cells in biliary cirrhosis are often not round but irregular, like jigsaw puzzle shapes

Robbins and Cotran Pathologic Basis of Disease

Primary biliary cirrhosis. A portal tract is markedly expanded by an infiltrate of lymphocytes and plasma cells. Granulomatous reaction to a bile duct undergoing destruction (florid duct lesion)

Robbins Basic Pathology

ductular reaction in a fibrotic septum

Robbins Basic Pathology

Primary Biliary Cirrhosis Two paths to end-stage liver disease

Primary biliary cirrhosis, end stage. This sagittal section demonstrates liver enlargement, nodularity indicative of cirrhosis, and green discoloration due to cholestasis

Robbins Basic Pathology

PRIMARY SCLEROSING CHOLANGITIS (PSC)

Primary Sclerosing Cholangitis (PSC)

Robbins and Cotran Pathologic Basis of Disease

Primary Sclerosing Cholangitis

Primary sclerosing cholangitis. A bile duct undergoing degeneration is entrapped in a dense, “onion-skin” concentric scar.

Robbins Basic Pathology

Drug/Toxin-Induced Cholestasis

CIRCULATORY DISORDERS

Robbins Basic Pathology

Impaired Blood Flow into the Liver

Hepatic Artery Inflow

Autopsy Pathology: A Manual and Atlas

Acute centrilobular hemorrhage and necrosis

Shock from acute blood loss or sepsis causes centrilobular hemorrhage and necrosis

Autopsy Pathology: A Manual and Atlas

Portal Vein Obstruction and Thrombosis

Impaired Blood Flow Through the Liver

Cirrhosis

Sickle cell disease

Disseminated intravascular coagulation

Eclampsia

Sickle cell crisis in liver

Passive Congestion and Centrilobular Necrosis

http://www.pathguy.com/lectures/nutmeg3.jpg

Autopsy Pathology: A Manual and Atlas

Centrilobular hemorrhagic necrosis (nutmeg liver)

Robbins Basic Pathology

Robbins Basic Pathology

Hepatic Vein Outflow Obstruction

Hepatic Vein Thrombosis (Budd-Chiari Syndrome)

Budd-Chiari syndrome. Thrombosis of the major hepatic veins has caused profound hepatic congestion.

Robbins Basic Pathology

Cholestasis and hepatic vein thrombosis (Budd-Chiari syndrome)

Autopsy Pathology: A Manual and Atlas

Sinusoidal Obstruction Syndrome

Venoocclusive disease

Toxic injury to sinusoidal endothelium

Damaged endothelial cells slough, leading to formation of thrombi that block sinusoidal flow

Endothelial damage allows red cells to spill into the space of Disse, causes proliferation of stellate cells and fibrosis of terminal branches of the hepatic vein

Sinusoidal obstruction syndrome. A central vein is occluded by cells and newly formed collagen. There is also fibrosis in the sinusoidal spaces.

Robbins Basic Pathology