Pathoma: Amyloidosis

Question 1

What are amyloid proteins?

Answer 1

Misfolded proteins that are deposited in the extracellular space

Question 2

Amyloid is usually in the ________ form.

Answer 2

beta-pleated sheet

Question 3

Describe primary amyloidosis.

Answer 3

Systemic deposition of AL amyloid derived from Ig; associated with plasma cell dyscrasias

Question 4

Describe secondary amyloidosis.

Answer 4

Systemic deposition of SA amyloid (derived from serum amyloid A) secondary to an inflammatory state (from, for example, autoimmune diseases or malignancies)

Question 5

Familial Mediterranean fever is an ______.

Answer 5

autosomal recessive disorder of neutrophils

Question 6

The two organs most commonly affected in amyloidoses are _______.

Answer 6

the heart (whose walls fill with amyloid) and the kidney (which gets nephrotic syndrome)

Question 7

Other than systemic amyloidoses, there are also _________.

Answer 7

localized amyloidoses, such as senile cardiac amyloidosis (serum transthyretin deposits in heart)

Question 8

The second-most common protein in the blood is _______.

Answer 8

transthyretin

Question 9

What type of amyloidosis is found in NIDDM (type II diabetes)?

Answer 9

Amylin deposits in the islet cells of the pancreas