pathology chapter 18 (pg 852-880) liver and gallbladder Flashcards
what is bilirubin the end production of
heme degradation
what coverts heme to biliverdin
intracellular heme oxygenase
what happens to bilirubin formed outside the liver
it is released and bound to serum albumin
how are bile salts formed
by the conjugation of bile acids with taurine or glycine
what is the major catabolic product of cholesterol
bile acids
describe unconjugated bilirubin in water
virtually insoluble and exists in tight complexes with serum albumin
what is cholestasis caused by
impaired bile formation and bile flow that gives rise to accumulation of bile pigment in the hepatic parenchyma
what is a characteristic lab finding with cholestasis
elevated serum alkaline phosphatase and gamma-glutamyl transpeptidase
what is morphologically common to both obstruction and non obstructive cholestasis
accumulation of bile pigment within the hepatic parenchyma
what is the most common cause of bile duct obstruction in adults
extra hepatic cholelithiasis (gallstones)
what is the most severe form of cholangitis
suppurative cholangitis
what is the histological hallmark of ascending cholangitis
influx of periductular neutrophils directly into the bile duct epithelium and lumen
where are bile plugs predominant with canalicular cholestasis
centrilobular canaliculi
what does hepatolithiasis lead to repeated bouts of
ascending cholangitis, progressive inflammatory destruction of hepatic parenchyma, and predisposes to biliary neoplasia
what kind of stones does hepatolithiasis have in distended intrahepatic bile ducts
pigmented calcium bilirubina stones
what are the morphological features of neonatal hepatitis
lobular disarray with focal liver cell apoptosis and necrosis
what disorder is defined as complete or partial obstruction of the lumen of the extra hepatic biliary tree within the first 3 months of life
biliary atresia
what are the salient features of biliary atresia
inflammation and fibrosing stricture of the hepatic or common bile ducts
what are the 2 main autoimmune disorders of the intrahepatic bile ducts
primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC)
what is primary biliary cirrhosis characterized by
nonsuppurative, inflammatory destruction of small and medium-sized intrahepatic bile ducts
who does PBC primarily effect
middle aged women with a female to male ratio of 9:1
what is the most characteristic finding for PBC
antimitochondrial antibodies
what do the antimitochondrial antibodies involved in PBC recognize
the E2 component of the pyruvate dehydrogenase complex
what lab values are elevated with PBC
asymptomatic with elevated serum alkaline phosphatase and gamma-glutamyltransferase
when is liver biopsy considered diagnostic for PBC
when a florid duct lesion is present
what is primary sclerosing cholangitis (PSC) characterized by
inflammation and obliterative fibrosis of intrahepatic and extra hepatic bile ducts with dilation of preserved segments
what is large duct inflammation seen in PSC similar to
that seen in ulcerative colitis
what is described as congenital dilations of the common bile duct
choledochal cysts
what are the primary abnormalities with fibropolycystic disease
congenital malformations of the biliary tree
what are von meyenburg complexes
small bile duct hamartomas
what does the thrombosis of intrahepatic portal vein radicals result in
sharply demarcated area of red-blue discoloration called infarct of zahn
what is the most common cause of small portal vein branch obstruction
schistosomiasis
what is the most common intrahepatic cause of blood flow obstruction
cirrhosis
what condition is the obstruction of two or more major hepatic veins that produces liver enlargement, pain and ascites
budd-chiari syndrome
describe the shape and coloration of the liver in budd-chiari syndrome
it is swollen and red-purple and has a tense capsule
what does sinusoidal obstruction syndrome arise from
toxic injury to the sinusoidal endothelium
what is sinusoidal obstruction syndrome characterized by
obliteration of the terminal hepatic venues by sub endothelial swelling and collagen deposition
what two things act synergistically to cause centrilobular hemorrhagic necrosis
combination of hypo perfusion and retrograde congestion
what is preeclampsia characterized by
maternal hypertension, proteinuria, peripheral edema and coagulation abnormalities
what do the periportal sinusoids contain with preeclampsia
fibrin deposits associated with hemorrhage into the space of disse, leading to periportal hepatocellular coagulative necrosis
what does the diagnosis of acute fatty liver of pregnancy rest on
biopsy identification of the characteristic diffuse microvesicular steatosis of hepatocytes
what is the primary treatment for acute fatty liver of pregnancy
termination of pregnancy
how does focal nodular hyperplasia appear morphologically
as a well-demarcated but poorly encapsulated nodule
describe the lesion color in focal nodular hyperplasia
generally lighter than the surrounding liver and is sometimes yellow indicating steatosis
nodular regenerative hyperplasia can lead to the development of what
portal hypertension
what are the most common benign liver tumors
cavernous hemangiomas
where are cavernous hemangioma generally located
directly beneath the capsule
describe the morphology of tumors resulting from HNF1-alpha mutations
often fatty and devoid of cellular or architectural atypia
what immunostain change is diagnostic for beta-catenin mutated hepatocellular adenomas
beta catenin usually shows nuclear translocation indicative of its activated state
what do most primary liver cancers arise from and what are they termed
arise from hepatocytes and termed hepatocellular carcinoma
what is the most common liver tumor of early childhood
hematoblastoma
what pathway is characteristically activated with hepatoblastomas
activation of WNT signaling pathway
what is the most common setting for emergence of HCC
chronic liver diseases
what are the most important underlying factors in hepatocarcinogenesis
viral infections and toxic injuries
what are the most common early mutation events for the emergence of HCC
activation of beta-catenin and inactivation of p53
describe the morphology of large cell change in HCC
show scattered hepatocytes, usually near portal tracts or septa, that are larger than normal hepatocytes and with large, often multiple, often moderately pleomorphic nuclei
how does fibrolamellar carcinoma typically present
single large, hard scirrhous tumor with fibrous bands coursing through it
define cholangiocarcinoma
malignancy of the biliary tree, arising from bile ducts within and outside of the liver
what form of cholangiocarcinoma is klatskin tumors associated with
extra hepatic forms
where are klatskin tumors located
at the junction of the right and left hepatic ducts
how do most extra hepatic cholangiocarcinomas present
firm, gray nodules within the bile duct wall
what type of tumors are cholangiocarcinomas usually
adenocarcinomas
what are most biliary tract diseases attributable to
cholelithiasis
what are pigmented gallstones complex mixtures of
insoluble calcio salts of unconjugated bilirubin along with inorganic calcium salts
where do cholesterol stones exclusively arise
gallbladder
describe pure cholesterol stones
pale yellow, round to ovoid, and have a finely granular, hard external surface
what happens to the color of cholesterol stones when the proportion of calcium carbonate, phosphates and bilirubin increase
the stones take on a gray-white to black color and may be lamellated
what is the primary complication of gallstones
acute calculous cholecystitis
what does acute calculous cholecystitis result from
chemical irritation and inflammation of a gallbladder obstructed by stones
what is acute calculus cholecystitis without stone involvement thought to result from
ischemia
where is the obstructed stone usually present in calculous cholecystitis
ni the neck of the gallbladder or the cystic duct
what is chronic cholecystitis usually associated with
cholelithiasis
what is the most common malignancy of the extra hepatic biliary tract
carcinoma
what is the most important risk factor for gallbladder cancer
gallstones
what are the 2 patterns of growth that carcinomas of the gallbladder show
infiltrating and exophytic
how does the exophytic pattern of gallbladder carcinomas grow
into the lumen as an irregular, cauliflower mass
what type of carcinomas are most carcinomas of the gallbladder
adenocarcinomas
