1.5 Amyloid Flashcards
What, generally, is amyloid? Where in tissues does it deposit?
A misfolded protein that deposits in the extracellular space, and damages tissues
What is the usual secondary structure of amyloid?
Beta-pleated sheet configuratio
What is the stain that is used to highlight amyloid? What technique is used to help visualize the stain?
Congo red
Polarized light causes it to show apple green birefringence
What is the type of amyloid that is depositied with primary amyloidosis?
AL amyloid, derived from Ig light chain
What are plasma cell dyscrasias?
Abnormalities of the plasma cells that produces systemic amyloid deposition
What is the amyloid that is deposited with secondary amyloidosis? What protein is this derived from?
AA amyloid
SAA protein, which is an acute phase reactant increased in chronic inflammatory states
What is the function of SAA? What, then, is secondary amyloidosis associated with?
Acute phase reactant that is increased in chronic inflammatory states
Associated with autoimmune diseases, malignancy
What is familial mediterranean fever? How does it present?
- AR Dysfunction of PMNs that causes them to be activated in the absence of infection
- Presents with episodes of fever and acute serosal infection
What is the relation between familial Mediterranean fever and amyloidosis?
Causes an increase in amyloid deposition
What is the most commonly involved organ with amyloidosis? What does this usually cause?
Kidney
-Nephrotic syndrome
What type of heart problems occur with amyloidosis?
Restrictive cardiomyopathy
What are the GI consequences of amyloid deposition?
- tongue enlargement
- Malabsorption
- HSM
How do you diagnose amyloidosis?
Tissue bx
Where are biopsies usually taken to diagnose amyloidosis?
Fat pad and rectum
What is the treatment for damage organs 2/2 amyloidosis?
Must be transplanted–amyloid cannot be removed
