Amyloidosis Flashcards
What is amylodoisis?
Group of diseases characterized by the deposition of an extracellular protein
How does amyloidosis stain?
Stains pink to red with H&E stain
Stains red with Congo red
Apple green birefringence of the amyloid is seen on the Congo red stain under polarized light
What is primary amyloidosis?
Has amyloid light chain (AL) amyloid, fibrillary protein is made up of kappa or lambda light chains.
May be seen in plasma cell disorders but most cases occur independent of other diseases
What is reactive systemic amyloidosis?
Has amyloid associated (AA) protein, precursor is serum amyloid A (SAA)
Can be seen in a wide variety of chronic diseases.
Examples:
RA, SLE, tuberculosis, bronchiectasis, osteomyelitis, IBD, and cancer
What is familial mediterranean fever?
Has AA amyloid.
This autosomal recessive disorder is characterized by recurrent inflammation, fever, and neutrophil dysfunction.
Gain of function mutations of pyrin are present
What is hemodialysis-associated amyloidosis?
Has Abeta2M type amyloid with precursor protein beta2-microglobulin.
May cause carpal tunnel syndrome and joint disease.
What is senile cerebral amyloidosis?
Alzheimer disease
Has Abeta type amyloid with fibrillary protein composed of beta-amyloid precursor protein (BetaAPP)
Gene located on chromosome 12
What is senile cardiac/systemic amyloidosis?
Has ATTR type amyloid with fibrillary protein composed of transthyretin.
Seen in men older than 70 and may cause heart failure
What is endocrine type amyloidosis?
Seen in medullary carcinoma of the thyroid (procalcitonin), adult-onset diabetes (amylin), and pancreatic islet cell tumors(amylin)
