Ch 23: Amyloidoses Q & A

Question 1

Diagnosis of amyloidosis requires what for confimation?

Answer 1

Tissue Biopsy

Question 2

All forms of amyloid stain (+) with this & show red-green birefringence when viewed under polarized light.

Answer 2

Congo Red

Question 3

Presentation of amyloidosis w/o any precceeding disease. Consists of variable region of immunoglobulin light chains & can be derived from either kappa or lamda polypeptides.

Answer 3

AL Amyloidosis (Primary)

Question 4

Occurs in patients w/ seemingly unrelated, persistant, inflammatory or neoplastic disorders. Ex. A patient w/ long standing RA, complains of swelling of eyelids, abdomen & ankles. Increase of what protein during inflammation causes what disease? What is the amyloidogenic form of this protein?

Answer 4

AA Amyloidosis (Secondary)
Serum Amyloid A to Apo Serum Amyloid A (amyloidogenic)

Question 5

Amyloidosis refers to a group of extracellular protein deposits that are best defined as what?

Answer 5

Proteins with a common morphologic properties

Question 6

Progessive cell & organ dysfunction will develop primarily as a result of what biological process in AA/AL amyloidosis?

Answer 6

Diminished blood supply to parenchymal cells.

Question 7

Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant disorder that is associated with a mutation in a gene for what protein?
This disorder usually manifests as paresthesias, w/ loss of temperature & pain sensation.

Answer 7

Transthyretin

Question 8

This is found in a woman with progressive neurodegenerative disease. Autopsy reveals spongiform encephalitis with brain amyloidosis

Answer 8

APrP (Amyloid Prion Protein)

Question 9

Derived from the protein observed in a congo red stain from a patient suffering from cognitive impairment. This protein gets deposited in the brain and cerebral vessels.

Answer 9

Beta-protein precursor

Question 10

Patients with Down syndrome are associated with Alzheimer’s disease. Autopsy would reveal cerebral deposits of what protein?

Answer 10

AB(beta)

Question 11

A patient presents with hypoalbuminemia, mild anemia & thrombocytopenia. Monoclonal peak is demonstrated by serum gel electrophoresis & a bone marrow aspiration demonstrates numerous atypical plasam cells. Proteinuria, polyuria & polydipsia on presentation. Renal biopsy would show deposits of what amyloid precursor proteins?

Answer 11

Immunoglobulin Light Chain

Question 12

Medullary carcinoma of the thyroid originates from C cells (APUD Cell) of the thyroid. The amyloid deposits are going to be most closely related to what serum protein?

Answer 12

Calcitonin

Question 13

Deposition of this protein in major joints of patients undergoing chronic renal dialysis is characterized by a destructive arthopathy

Answer 13

B2-Microglobulin

Question 14

This is a heterogenous disorder characterized by reduced sensitivity to insulin & inadequate secretion of insulin from the pancreas. What is the disease? Islet amyloid polypeptide is also known as?

Answer 14

Diabetes Mellitus Type 2

Amylin