B5.025 Prework 1 Patterns of Hepatic Injury

Question 1

4 major patterns of hepatic injury

Answer 1

degeneration
intracellular accumulation
liver cell death- necrosis and apoptosis
inflammation

Question 2

list some common materials that accumulate in cells

Answer 2

bile
fat
iron
copper
protein in RER
lipofuscin

Question 3

features of bile accumulation

Answer 3

feathery degeneration of cytoplasm

due to emulsifying properties of bile

Question 4

common causes of fat accumulation

Answer 4

obesity, alcohol, diabetes

Question 5

what is Mallory hyaline

Answer 5

clumped, amorphous cytokeratin filaments
dark pink clumps in ballooning hepatocytes
often seen in alcoholics, but nonspecific

Question 6

features of iron accumulation

Answer 6

hemochromatosis (genetic mutation)
refractile coloration and canalicular distribution (where membranes of hepatocytes meet)
stains blue on Perls Iron stain

Question 7

what is “bronze diabetes”

Answer 7

genetic hemochromatosis

hyperpigmentation + diabetes + cirrhosis = classic triad

Question 8

features of hereditary hemochromatosis

Answer 8

autosomal recessive
classic triad
increased serum iron, increased transferrin, and increased serum ferritin

Question 9

treatment of hemochromatosis

Answer 9

weekly phlebotomy and deferoxamine

Question 10

mutations associated with hemochromatosis

Answer 10

HFE protein regulates hepcidin

chromosome 6: C282Y (90%), H63D (10%), S65C

Question 11

pathology of hereditary hemochromatosis

Answer 11

inappropriately high intestinal iron absorption due to defective hepcidin
results in excess body iron stores
slow accumulation but typically tissue damage by age 30 and cirrhosis by age 40
male gender, hep C, and alcohol provoke disease expression

Question 12

histo features of A1AT def

Answer 12

PAS-diastase resistant gobules in hepatocytes

globules are aggregations of abnormally folded proteins

Question 13

presentation of A1AT def

Answer 13

liver: hepatitis, cirrhosis, HCC
lung: early onset emphysema, chronic bronchitis, bronchiectasis, and asthma

Question 14

genotypes of A1AT def

Answer 14

PiMM: normal levels
PiZZ: 10% normal A1AT levels, 1 in 7000
PiMZ: intermediate levels

Question 15

histologic features of Wilson disease

Answer 15

copper aggregated on rhodanine stain

Question 16

characterize Wilson disease

Answer 16

rare autosomal recessive disorder involving mutation of copper-transporting protein (ATPase) leading to defective conjugation of copper to ceruloplasmin
onset: 10-30’s
increased copper absorption in the gut and decreased excretion via bile leads to accumulation-esp in basal ganglia, liver, kidney, cornea

Question 17

treatment for wilsons

Answer 17

penicillamine for copper chelation

Question 18

patterns of liver cell death

Answer 18

apoptosis- eosinophilic bodies in viral hepatitis
focal or interface periportal necrosis in many forms of hepatitis
zonal toxic necrosis- due to toxins, ischemia
submassive necrosis- toxic or viral hepatitis

Question 19

lipofuscin accumulation

Answer 19

associated with aging
not harmful
differentiate from other accumulations with staining properties