Amyloidosis Flashcards
How is the accumulation of protein found in an amyloid organized?
In beta pleated sheets that forms fibrils
What kind of stain and imaging are good for amyloidosis?
Congo red stain, polarized light which would yield an “apple green” birefringence
Where does the AL protein originate?
Monoclonal B-cell proliferation–> Plasma Cells–> Ig light chain*–> AL protein
Where does the AA protein originate?
Chronic inflammation–> macrophage activation–> IL-1, IL-6 released–> Liver–> SAA*–> AA
Which amyloid and its precursor result from a production of normal amounts of mutant protein?
Transthyretin–> ATTR protein
What is the amyloid protein in hemodialysis associated amyloidosis?
Beta 2 microglobulin, deposited in synovium, joints, and tendon sheaths
Which light chain is chiefly responsible for immunocyte dyscrasias amyloidosis?
Lambda light chain
Reactive systemic amyloidosis is the result of what type of amyloid?
AA resulting from chronic inflammation e.g. RA, TB
What are the amyloid proteins in familial Mediterranean fever?
SAA, AA
What are the amyloid proteins for familial amyloidotic neuropathy?
tranthyretin, ATTR
What are the amyloid protein for senile cardiac amyloidosis?
tranthyretin, ATTR
What effect can amyloidosis have on the kidney?
It can cause nephrotic syndrome with severe proteinuria
