AA Amyloidosis

Question 1

Pathophysiology of AA amyloidosis

Answer 1

Inflammation–>Hepatic production of SAA protein–>Inadequate breakdown and/or excessive accumulation–>Accumulation of AA amyloid

Question 2

What are additional methods to look for amyloid after it seems to show up under a normal light-microscope

Answer 2

Congo red stain

Electron microscopy

Question 3

Misfolded protein accumulation leads to

Answer 3

Fibril formation

Question 4

What leads to AL protein formation

Answer 4

Acquired mutation leads to B cell proliferation, then plasma cells, then Ig light chains aggregate with limited proteolysis

Question 5

What leads to AA protein formation

Answer 5

Chronic inflammation leads to IL-1, IL-6 release, these act on liver and tell it to release SAA proteins which aggregate due to limited proteolysis/excess production

Question 6

What leads to ATTR protein formation

Answer 6

Mutation leads to mutant transthyretin which aggregates

Question 7

What is serum staining used for

Answer 7

To discriminate the particular amyloid type. Brown means AA