Amyloidosis

Question 0

AL

Answer 0

Primary- deposition of proteins form ig light chain. Seem in plasma cell disorders and multiple myeloma . Systemic - nephrotic syndrome, restrictive cardiomyopathy, enlarged tongue, easy bruising, malabsorption, hepatomegaly , neuropathy

Question 1

Structure and stain

Answer 1

Beta pleated sheet

Congo red stain- apple green birenfringence under polarized light

Question 2

Aa

Answer 2

Secondary- seen with chronic diseases. Chrons, UC,RA, chronic infection, malignancy. You get a lot of SAA (serum amyloid a)

Question 3

B2 a microglobulin fibrils

Answer 3

Dialysis- related seen in pts with esrd. Presents with carpal tunnel syndrome and other joint issues

Question 4

Mutated transthyretin

Answer 4

Restrictive cardiomyopathy . Heritable African Americans

Question 5

Normal or wild type transthyretin

Answer 5

Asymptomatic - age related or senile systemic . In myocardium and other sites but just over a long long time

Question 6

AB sheets

Answer 6

Alzheimer’s - from beta amyloid precursor protein on chrom 21 ( downs early Alzheimer’s)

Question 7

Presents with aa and fevers and are from Greece

Answer 7

Familial Mediterranean fever- ar, you have bad neutrophils and you get fever and acute serosal inflammation that can look like appendicitis or mi. High SAA during the attack and you get the deposition of aa

Question 8

Tumor cells in a amyloid background

Answer 8

Medullary carcinoma of the thyroid. Calcitonin deposits im tumor

Question 9

Deposition in type 2 dm

Answer 9

Amylin in islets of pancreas