Amyloidosis Flashcards
What is amyloid?
- Misfolded protein that deposits in extracellular space
- Damages tissues
What kind of protein is amyloid?
Beta-pleated sheet configuration
- Multiple proteins can be referred to as amyloid although they are all deposited in a particular way
How does amyloid stain?
- Congo red staining
- Apple-green birefringence under polarized light
Where does amyloid commonly deposit?
Around blood vessels
How can systemic amyloidosis be divided?
Into primary and secondary types
What protein is deposited in primary amyloidosis?
AL amyloid
What is AL amyloid derived from?
Ig light chain
What is primary amyloidosis associated with?
Plasma cell dyscrasias
- Overproduced light chain leaks out into blood, can become misfolded and deposit within tissue
Where are the light chains located in relation to the heavy chains?
Outside
What protein is deposited in secondary amyloidosis?
AA amyloid
What is AA amyloid (secondary amyloidosis) derived from?
SAA
What is SAA?
What is it increased in? (what diseases/states)
SAA is an acute phase reactant and a family of apolipoproteins associated with high-density lipoprotein (HDL) in plasma
- Chronic inflammatory states (lupus, rheumatoid, crohn’s)
- Malignancy
- Familial Mediterranean fever
What is familial Mediterranean fever?
How does it present?
- Dysfunction of neutrophils
- Presents with episodes of fever and acute serosal inflammation (appendicitis, pleuritis, pericarditis)
What kind of amyloidosis does Familial Mediterranean fever present with?
Secondary amyloidosis
- High SAA during attacks deposits as AA amyloid
How is Familial Mediterranean fever inherited?
Aut Recessive
