Clinicopathology of Hereditary liver disease Flashcards
(66 cards)
Hereditary hemochromatosis presentation
- very tanned (tan in wintertime)
- abnormal LFTs
- increased ferritin levels
primary iron overload
Hereditary hemochromatosis
secondary iron overload
alcohol or NAFLD/NASH, transfusion related
Hemochromatosis
disorder of iron metabolism that leads to iron overload
Hereditary hemochromatosis mechansism
body absorbs too much iron from the diet, excess iron stored in the liver, skin, heart, pancreas, testes and joints, severe tissue damage
where is iron deposited in HH
liver, skin, heart, pancreas, testes and joints
histology of HH
iron overload, fibrosis, cirrhosis
total body iron stores by age in HH
men present earlier than women
women have menstrual cycle which naturally decreases iron stores - will present about 15 years later
how do we detect iron overload?
can see on MRI
which organ does not acculumate iron?
spleen - therefore color can be used as a reference on MRI imaging
hepatocytes with Fe overload
yellowish brown, refractive granules
what stain tests for iron?
Prussian blue
HH histology
low power: areas of brownish discoloration over the liver
high power: portal area in the central portion of the field, periportal hepatocytes with refeactile yellow brown pigment
where does iron deposition in the liver start?
periportal areas - zone 1!!!! other disease are zone 3 due to lack of blood flow, but zone 1 here because liver thinks it wants the iron
untreated HH can lead to
cirrhosis
which cancer can arise from hemochromatosis with cirrhosis?
hepatocellular carcinoma - common because iron is toxic, so it can cause mutations
pathology can show larger nodules with no accumulation of iron, with cellular atypic and thicker cell plates
do hepatocellular carcinomas accumulate iron?
no because cirrhotic regenerative nodule has not had time to accumulate iron
non-genetic mechanism for iron overload
secondary
transfusion associated iron overload, or patients on chronic transfusion regiments (sickle cell, hemolytic anemias, etc)
difference between primary iron deposits and secondary iron deposits
primary includes iron deposits in the liver cell hepatocytes
secondary has iron in the kupferr cell macrophages, trying to clear it out, and then progresses to hepatocytes as overload increases
difference between primary iron deposits and secondary iron deposits
primary includes iron deposits in the liver cell hepatocytes
secondary has iron in the kupferr cell macrophages, trying to clear it out, and then progresses to hepatocytes as overload increases
what is the hepatic iron index
it normalizes to a patients age to determine if there is a hereditary cause or not
how can you tell if patient has hemolytic anemia on liver biopsy?
abnormal shaped cells in sinusoidal spaces (sickle cell)
hemochromatosis treatment
phlebotomy - removal of iron to deplete stores
unless patient is anemia or has a blood disorder - chelating agents
prime protein of iron absorption
hepcidin
