Pathology Flashcards
This is a sudden liver illness that is associated with encepthalopathy within 6 months after the initial Dx.
Acute liver failure.
This form of acute liver failure is when the encephalopahy develops within 2 weeks of the onset of jaundice.
Fulminant liver failure
This form of acute liver failure is when the enceopahlopathy develops within 2 months of the onset of jaundice.
Sub-fulminant liver failure
What is the main pathological change in acute liver failure?
massive hepatic necrosis
Which drug accounts for 50% of cases of acute liver failure?
acetaminophen
Which hepatitis viruses can lead to acute liver failure?
HAV, HBV, and HCV
This is the most common cause of hepati failure and is the end point of relentless long term hepatitis ending in cirrhosis.
Chronic liver disease
This is the condition when the hepatocytes remain viable but are unable to perform normal metabolic fxn.
Hepatic dysfxn without overt necrosis
Generally, this is the failure of the hepatocytes to perform their homeostatic functions.
Hepatic failure.
The elevation of what substance in the blood and CNS contributes to hepatic encephalopathy?
ammonia levels
What are some common neurological manifestations of hepatic encephalopathy?
Rigidity, hyperreflexia, and asterixis
Which cells swell in the brain that may not just be a minor morphological change?
astrocytes
True or False: encephalopathy is reversible if the underlying hepatic condition is corrected.
True!
This is the condition where there is renal failure in individuals with severe chronic liver disease.
Hepatorenal syndrome.
What is the main contributor that causes hepatorenal syndrome?
Decreased renal blood flow
What will change with the urine output in hepatorenal syndrome?
It’ll decrease
In hepatorenal syndrome, which 2 substances increase in teh blood?
BUN and creatinine
This condition is characterized by the triad of chronic liver disease, hypoxemia, and IVPD.
hepatopulmonary syndrome (HPS)
In HPS, which mediator is produced more in the lung?
NO
What are the 2 main manifestations for HPS?
orthodeoxia and platypnea
These are delicate bands or broad scars linking portal tracts with 1 another in cirrhosis.
Bridging fibrous septa
These are nodules containing hepatocytes encircled by fibrosis in cirrhosis.
Parenchymal nodules
True or False: focal injury with scarring consitutes cirrhosis.
FALSE. the parenchymal injury and fibrosis is diffuse.
This condition is the triad of hepatocyte death, ECM deposition, and vascular reorganization.
Cirrhosis
What do you lose in cirrhosis which leads to intrasinusoidal HTN?
fenestrations of sinusoidal endothelial cells.
Which cells proliferate and are activated, which is the predominant mechnaism of fibrosis in cirrhosis?
hepatic stellate cells
Hepatic stellate cells are activated into what cells as a result of PDGFR-beta?
myofibroflasts
Portal HTN, hypoalbuminemia, and hyperaldosteronism can lead to what condition where there is excess fluids in the peritoneal cavity?
Ascites
True or False: the concentration of solutes in ascites is similiar to that of the blood.
True.
This is the condition when a rise is portal system pressure causes reversal of the flow from portal to the systemic circulation.
Portosystemic venous shunts
What are the 3 main varices that can result from portsystemic venous shunts?
hemorrhoids, esophageal varices, and caput madusae
Long-standing congestion can cause enlargement of what organ?
Spleen
What secondary things might be caused when a pt has congestive splenomegaly?
hypersplenism with various cytopenias
Bilirubin overproduction, hepatitis, and bile obstruction can all cause what condition?
Jaundice.
True or False: unconjugated bilirubin is insoluble in water at physiological pH and cannot be excreted in the urine.
True
This is the hemolytic disease of the newborn as a result of an accumulation of unconjugated bilirubin in the brain.
Kernicterus
So what form of bilirubin can be excreted in the urine because it’s loosely bound to albumin?
Conjugated form
Almost every newborn develops what kind of hyperbilirubinemia?
mild unconjugated hyperbilirubinemia
Why can mild unconjugated hyperbilirubinemia in infants be exasterbated by breastfeeding?
cuz theres bilirubin-deconjugating enzymes in boob milk
This genetic hyperbilirubinemia is when a UGT1A1 absence causes colorless bile and serum unconjugated bilirubin reaches a very high level.
Crigler-Najjar Syndrome Type I
This genetic hyperbilirubinemia is a less severe form of CNS type I, where the UGT1A1 is reduced and can only form monoglucuronidated bilirubin.
Crigler-Najjar Syndrome Type II
What does a pt with Crigler-Najjar Syndrome Type I die of in 18 months?
Kericterus
This genetic hyperbilirubinemia is a benign condition where there is a mild, fluctuating hyperbilirubinemia because hepatic bilirubin-glucuronidating activity is only 30% of normal.
Gilbert syndrome
This AR hyperbilirubinemia is characterized by chronic conjugated hyperbilirubinemia from a defect in hepatocellular excretion of bilirubin glucuronides from the canalicular membrane.
Dubin-Johnson Syndrome
Why is the liver darkly pigmented in DJS?
from inclusions of bilirubin glucuronides in the cytoplasm of hepatocytes
This rare hyperbilirubinemia is characterized by asymptomatic conjugated hyperbilirubinemia, normal liver morphology, and chronic jaundice.
Rotor Syndrome.
This is a pathological condition of impaired bile formation and bile flow, causing an accumulation of bile pigment in the hepatic parenchyma
Chelestasis
Whether it’s extra or intra-hepatic causation of cholestasis, what 2 serum enzymes are elevated?
alkaline phosphatase & γ-glutamyl transpeptidase (GGT)
What is the appearance of the hepatocytes when dropets of bile pigment accumulates within them?
Fine and foamy appearance (feathery degeneration)
Progressive Familial Intrahepatic Colestasis (PFIC) has what type of genetic inheritance?
AR
In PFIC-1, when do u get liver failure?
before adulthoold
What is the mutated gene in PFIC-1?
ATP8B1
True or False: there is decreased GGT activity in PFIC-1.
False. it’s pretty much normal
What is the structure that is mutated in PFIC-2?
bile salt export pump
Which gene is mutated to cause PFIC-2?
ABCB11
True or False: there are normal levels of GGT in PFIC-2.
True
When do pt’s progress to cirrhosis in PFIC-2 in their life?
in the first decade of life.
Which cancer are PFIC-2 pt’s at risk for?
cholangiocarcinoma
Which gene is mutated to cause a defect in the protein MDR3 in PFIC-3?
ABCB4
True or False: there are normal levels of GGT in PFIC-3.
False. They are high.
Why are serum GGT levels high in PFIC-3?
because of an absence of secreted physphatidylcholine in the bile.
What is the cancer associated with HBV?
primary hepatocellular carcinoma
This the the name for the empty cytoplasm with scattered eosinophilic remnants in acute hepatitis.
Ballooning degeneration
Which calls undergo hypertrophy and hyperplasia as an inflammatory response in acute hepatitis?
Kupffer cells
What is the hallmark of chronic hepatitis?
deposition of fibrous tissue
This is a chronic and progressive form of hepatitis which is attributed to T-cell-mediated autoimmunity.
Autoimmune hepatitis
What 3 things can trigger an autoimmune hepatitis condition?
viral infections, drugs, and herbal products.
What are elevated in serum panels for autoimmune hepatitis?
IgG autoantibodies
This type of autoimmune hepatitis has a presence of ANA, SMA, AAA, and anti-SLA/LP antibodies.
Type 1 autoimmune hepatitis
What 2 things are present in type 2 autoimmune hepatitis?
ALKM-1 and ACL-1
Which WBC is present in the portal tracts and hepatic lobules in autoimmune hepatitis?
Plasma cells
Chronic drug-induced liver disease is histologically indistinguishable from what other disease?
chronic viral hepatitis
So how can u distinguish the 2?
serological markers for viral or drug induced hepatitis
What is the most common cause of drug induced acute liver failure?
Acetaminophen
This is a rare and potentially fatal syndrome of mitochondrial dysfxn of the liver, brain, and elsewhere after administration of aspirin
Reye syndrome
What types of pt’s are at risk for reye syndrome?
kids
What is the histological appearance of the hepatocytes in reye syndrome?
extensive accumulation of fat droplets within hepatocytes
Hepatic steatosis, alcoholic hepatitis, and cirrhosis are all factors which make up which condtion?
alcholic liver disease
This is the condtion where there are clear macrovesicular globules that compress and displace the hepatocyte nucleus.
Hepatic steatosis
What is the morphology of the liver in hepatic steatosis?
leargy, heavy, yellow, and greasy
This condition is where there is hepatocyte swelling and necrosis from chemical injruy to hepatocytes, typically after binge drinking.
Acoholic hepatitis
What are the names of the scattered eosinophilic cytoplasmic clumps of cytoketatin within hepatocytes in alcholic hepatitis?
Mallory bodies
Which WBC surrounds the mallory bodies in alcholic hepatitis?
Neutrophils
True or False: in cirrhosis, the liver initially is large and fatty but then turns into a brown shrunken nonfatty light liver.
True.
What type of tissue gets laid down in cirrhosis to cause the “hobnail” appearance on the surface of the liver?
fibrous tissue
True or False: men are more susceptible to hepatic injury than women.
False. Women > men
True or False: African Americans are less susceptible to hepatic injury than men.
False. They are more.
Which gene can be mutated to cause a lack of intolerance to alcohol? (lol Asians)
ALDH
These are a group of condtions which have a presence of hepatic steatosis in individuals who do not consume alcohol or do so in small quantities.
Nonalcoholic fatty liver disease (NAFLD)
What is the progressive form of NAFLD called?
Non-alcoholic steatohepatitis (NASH)
Where do u see fibrosis in NASH?
portal tracts aroudn the terminal hepatic venules
This condition is characterized by excessive accumulation of body iron, most of which is depositied in the liver and the pancreas.
Hemochromatosis
What is the mechanism for the overload of Fe to cause hemochromatosis?
excessive iron absorption
This condtion is an accumulation of Fe in tissues, which occurs because of parental administration of Fe usually in the form of transfusions.
hemosiderosis
Which protein, which is encoded by the HAMP gene, is the main regulator of Fe absorption, which can cause hemocrhomatosis?
hepcidin
What does hepcidin do tot he cellulear Fe efflux channel ferroportin (FPN) to cause hemochromatosis?
binds to it and internalizes and proteolysis it
So if you lack hepcidin, what happens to Fe levels?
overload cuz the Fe efflux channels are never internalized and constantly spit Fe into the plasma.
What is the morphology of the liver in hemochromatosis?
golden-yellow hemosiderin granules are in the cytoplasm of hepatocytes. fibrosis. cirrhosis in a intensly pigmented liver
What is the triad of Sx in hemochromatosis?
hepatomegaly, skin pigmentation, and DM
This condition is an AR disorder where there is impaired Cu excretion into bile and a failure to incorporate Cu into ceruloplasmin.
Wilson disease
Where does Cu accumulate in Wilsons?
liver, brain, and eye mainly.
What is the fxn of ceruloplasmin?
used in the blood to carry Cu to the liver where it is degrades and the Cu is excreted in the bile.
What is the morphology of the lvier in Wilson disease?
steatosis with vacuolated nuclei (glycogen or water) and occasionally focal hepatocyte necrosis
Though Wilsons can look axactly like acute viral hepatitis, what is the different change in wilsons that isnt in acute viral hepatitis?
fatty change.
Will ceruloplasmin levels in Wilsons be high or low?
low
What is the name of the ring that forms aorudn the eyes in Wilsons?
Kayser-Fleisher rings
The defciciency in this protein can cause pulmonary emphysema as well as liver disease.
Alpha 1-antitrypsin (A1AT)
What are the types of inclusions in hepatocytes in A1AT deficiency?
Round-oval cytoplasmic globular inclusions, which are acidophilic, PAS+
At this age, 10-20% of individuals present with cholestatic jaundice in A1AT deficiency.
Newborns
This form of hepatitis has focal liver cell apoptosis and necrosis, giant cells, and a parenchymal pattern of injury, typicall in kids.
Neonatal hepatits
This intrahepatic biliary tract disease is characterized by a course fiberous septa that subdivide the liver in a jigsaw like pattern, and has feathery degeneration and formation of bile lakes.
Secondary biliary tract disease
What is the main cause of secondary biliary tract disease?
bile duct obstruction.
Which levels of serum proteins are high in secondary biliary tract disease?
conjugated hyperbiliruninemia, increased alk phos, bile acids, and cholesterol.
What are the Sx to secondary biliary tract disease?
prutitus, jaundic, malaise, dark urine, light stools, hepatosplenomegaly.
This intrahepatic biliary tract disease is characterized by autoimmune destruction of the bile ducts.
Primary biliary cirrhosis
Since the bile ducts are destroyed in primary biliary cirrhosis, what histological changes can u see?
noncaseating granulomatous inflmmation
This intrahepatic biliary tract disease is characterized by an uknown etiology that cuases periductal portal tract fibrosis, with an onion-like appearance of the ducts.
Primary Sclerosis Cholangitis
“Penn State Cries because of onions”
also, penn state is a cult. srsly.
In this condition, the larger ducts of the intrahepatic biliary tree are segmentally dilated and many contain inspissated (thickened) bile.
Caroli disease.
In this condition, R sided cardialc decompensation can cause an enlarged, tense, and cyanotic liver.
Passive congestion of the liver
In this condition, L sided cardiac failure or shock leads to hepatic hypoperfusion and hypoxia, leading to ischemic coagulative necrosis of hepatocytes.
Centrilobilar necrosis
In this condtion, a combination of hypoperfusion and retrograde congestion causes a mottled appearance reflexing hemorrhage and necrosis in the centrilobular regions (nutmeg).
Centrilobular hemorrhagic necrosis
This condition is when sinusoidal dilation occurs when efflux of hepatic blood is impeded and can lead to blood-filled cystic spaces
Peliosis hepatis
Peliosis hepatis is associated with what other conditions?
Cancer, TB, AIDS, post-transplant immunodeficiency, steroids, and rarely oral contraceptives.
This condition results from obstruction of 2 or more major hepatic veins producing liver enlargement, pain, and ascites, lading to hepatic dmg.
Budd-Chiari syndrome.
this is the condition where some of the small veins of the liver are obstructed, typically happeining within 3 wks of marrow transplantation.
Sinusoidal obstruction syndrome.
In this condition, lympohocytes attack the epithelial cells of the liver, resulting in hepatitis with necrosis of hepatocytes and bile duct epithelial cells.
acute graft-vs-host disease.
In acute graft-vs-host disease, when does the Sx occur after transplant?
10-50 days.
In chronic graft-vs-host disease, how many days after transplant can u get portal tract inflammation and bile duct destructin?
> 100 days
this is so horrible. omg im dying.
This is the condition which is characterized by maternal HTN, proteinuria, peripheral edema, coagulation abnormalities, and varying degrees of disseminated intravascular coagulation.
Pre-eclampsia
This subsection of pre-eclampsia is characterized by hemolysis, eleveated liver enzymes, and low platelets
HELLP
Women in their 3rd trimester are at risk for what condition, which can lead to hepatic failure, coma, and death?
Fatty liver
Case: a chick on oral contraceptives is having a cholecyststectomy and you notice a well-demarcated but poorly encapsulated nodule about 6cm in diameter. What is is?
focal nodular hyperplasia
Case: A young woman presents with cords and sheets of cells in an adenoma biopsy. Which complication could be life threatening for her?
Intraperitoneal Hemorrhage during pregnancy
This benign neoplasn is when a tumor of a blood vessel and appears as a dsicrete red/blue, soft nodule usually <2cm in diameter.
Cavernous hemangioma
THis benign neoplasm derives from hepatocytes, occurs more frequently in women who use contraceptives, and are yellow-tan nodules anywhere in the hepatic substance.
Hepatic adenomas
This is the most common liver tumor of young childhood.
Hepatoblastoma
What are the 3 forms of hepatocellular carcinomas?
unifocal mass, multifocal mass, diffusively infiltrative cancer.
What are the 3 risk factors for hepatocellualr carcinomas?
chronic hepatitis, cirrhosis, anatoxins from aspergillus
This is the 2nd most common HCC of the biliary tree, arising from bile ducts in and out of the liver.
Cholangiocarcinoma
This type of gallstone is made of crystalline cholesterol monohydrate, and occur with MURICANS.
Cholesterol stones
This type of gallstone is made of bilirubin Ca++ salts, and occur in non-muricans.
Pigment stones
Fat fertile females over forty get which type of gallstones?
Cholesterol stones.
What genetic disorder can u have mutated to cause cholesterol stones?
ATP-binding cassette (ABC) transporters
Which medical conditions predispose u to pigmented stones?
chronic hemolytic syndromes, biliary infections, ileal disease, CF
What are the 4 ways u can form cholesterol stones?
supersaturation, hypomotility, crystal nucleation, accetion within gallbladder mucous layer
the release of what enzymes cause unconjugated bilirubin to increase, leading to pigmented stones?
microbial beta-glucoronidases
What is the morphology of cholesterol stones?
pale yellow, round, glistening, can be severeal cm
What is the morphology of black pigmented stones?
small, numerous, hard, crumble, radiopaque (cuz of Ca++ and phosphate)
Under what conditions do u get brown stones?
in infected intrahepatic or extrahepatic ducts (black stones are when its sterile)
What is the morphology of brown pigmented stones?
pure Ca++ salts of unconj bilirubin, laminated, soft, greasy, radiolucent.
This type of acute cholecystitis is caused by chemical irritation and inflammation from gallbaldder obstruction in the absence of bacterial infection.
Acute calculous cholecystitis
In what pt population is Acute calculous cholecystitis common?
diabetics
This type of acute cholecystitis is caused by ischemia.
Acute acalculous cholecystitis
What is unique to the cystic artery to cause acute acalculous cholecystitis?
it has no collateral citculation, so anything that causes inflammation can obstrcut the duct.
What is the mophology of the gallbladder in acute cholecystitis?
large, thickened/edematous/hyperemic walls.
What are the Sx of acute cholecystitis?
progressive RUQ pain + fever, anorexia, tachycardia, N/V
True or False: in chronic cholecystitis, there still has to be obstruction of the gallbladder outflow to cause the pathology.
False. it is not a requisite.
What happenes to bile to cause chronic cholecysitits?
supersaturation
What are the outpouchings of the mucosal epithelium through the wall called in chronic cholecystitis?
Rokitansky-Aschoff sinuses
Extensive dystropic calcification can lead to a really hard gallbladder in chronic cholecystitis. What is this called?
porcelain gallbladder
This is the term for the presence of stones within the bile ducts of the biliary tree
Choledocholithiasis
Which groups of people get Choledocholithiasis more commonly?
Asians
lol ella
This is the term for the bacterial infection of the bile ducts.
Cholangitis
What are the 2 main etiologies for cholangitis?
Choledocholithiasis and Biliary strictures
Which gram stain are the bacteria that cause cholangitis?
gram -
This is the partial or complete extrahepatic biliary tree obstruction within the first 3 months of life.
Biliary atresia
The fetal form of biliary atresia comes from the aberrant development of what structure?
Extrahepatic biliary tree
The more common perinatal form of biliary atresia may be assocaited with what 2 factors?
viral infection or autoimmunity
Type III of biliary atresia is incorrectable because the obstruction of the bile ducts is above what?
The porta hepatis
What is the most common malignancy of the biliary tract?
gallbladder adenocarcinoma
What is the morphology of infiltrating gallbladder carcinomas?
poorly defined area of diffuse thickening and induration, deep ulceration, scirrhous
What is the morphology of exophylic gallbladder carcinomas?
growth into lumen, cauliflower mass, necrosis, hemorrhagic and ulceration of the fundus and neck.
Agensis of the pancreas can be a result of a mutation in which gene?
PDX1
What is the most common anomaly of the pancrea, where there isnt a fusion of the detal duct system of the dorsal and ventral pancreatic primordials.
Pancreas divisum.
Where does the pancreas drain now in pancreas divisum?
through the doral duct (of santorini) and small-baliber minor papilla
What is it called when pancreatic tissues get depositied in other organs?
ectopic pancreas
What is called when pancreatic tissue encircles the duodenum and can cause duodenal obstruction?
Annular pancreas
This condition is when there is inflammation with associated microvascular leakage, fat necrosis, proteolytic destruction of pareatic parencyma, and interstitial hemorrhage
Acute pancreatitis
What is the morphology of acute necrotizing pancreatitis?
red-black hemorrhage with yellow-white chalk foci of fat necrosis
What are the Sx to acute pancreatitis?
abdominal pain, anorexia, N/V
What serum enzymes are increased in acute pancreatitis?
amylase and lipase
This is inflammation of the pancreas with irreversible destruction of the exocrine parenchyma, fibrosis, and destruction of the endocrine parenchyma.
Chronic pancreatitis
What is the msot common cause of chronic pancreatitis?
long term alcohol abuse
What is the morphology of chronic pancreatitis?
parenchymal fibrosis, reduced # and size of acini with relative sparing of the islets of Langerhands, and variable dilation of pancreatic ducts.
What is the autoimmune destruction of the pancreas called?
Lymphoplasmacytic sclerosing pancreatitis
Case: you’re doing a cholecystectomy and find a unilocar, thin-walled, glistening cyst on the pancreas. You also notice it has a thin fiberous capsule around it. What type of cyst is it?
Congenital cyst
What 2 AD diseases contribute to congenital cysts?
polycystic kidney disease and von Hippel-Lindau
This is a collection of necrotic-hemorrhagic material with enzymes but has NO epithelial lining on the pancreas.
Pseudocyst
Are congenital cysts and pseudocyts neoplastic or non-neoplastic?
non-neoplastic
These are benign cysts of glycogen-rich cuboidal cells surrounding small cysts containing clear, thin, straw-colored fluid .
serous cystadenomas
Serous cystadenomas look like what type of fruit?
Grapefruit
These cysts are assocaited with invastive carcinomas, arise in the body or tail, are large, and filled with thick mucin and lined by columnar muci-producing epithelium.
Mucinous cystic neoplasms
These are mucin-producing intraductal neoplasms, and are usually in the head of the pancreas.
Intraductal Papillary Mucinous Neoplasms (IPMNs)
These are large, well-circumscribed masses with solid and cystic componenets, which grow in solid sheets in the pancreas.
Solid-pseudopapillary neoplasm
Which genetic pathway does Solid-pseudopapillary neoplasms usually involve?
B-catenin/APC pathway
This is the condition where non-neoplastic epithelium develops into noninvasive lesions, which can then turn into invasive carcinomas of the pancreas.
Pancreatic cancer (infiltrating ductal adenocarcinomas of the pancreas)
What is the gene mutation to cause pancreatic cancers?
KRAS > p16/CDKN2A > TP53 > others
What is a big risk factor for pancratic cancer?
Smoking
What is the morphology of pancreatic cancer?
hard, stellate, gray-white, poorly defined masses that invade
This is the cancer that had prominant acinar cell differentiation, formation of zymogen granules and production of exocrine enzymes.
acinar cell carcinoma
This is the rare childhood cancer of the pancreas where there are squamous cell islands admixed with acinar cells.
Pancreatoblastoma
