Amyloidosis

Question 1

What is amyloidosis?

Answer 1

Abnormal aggregation of proteins (or their fragments) into β-pleated sheet structures → damage and apoptosis

Question 2

What is affected with AL (primary) amyloidosis?

Answer 2

Multiple organ systems (renal, cardiac, hematologic, GI, and neurologic)

Question 3

What causes the symptoms seen with AL amyloidosis?

Answer 3

Deposition of proteins from Ig light-chains

can be seen with a plasma cell disorder or with multiple myeloma

Question 4

AA (secondary) amyloidosis presents similar to primary amyloidosis. When is secondary amyloidosis seen?

Answer 4

With chronic conditions (RA, IBD, spondyloarthropathy, protracted infection)

Question 5

What is seen with dialysis-related amyloidosis?

Answer 5

β(2)-microglobulin fibrils…may present as carpal tunnel syndrome

Question 6

What are two common types of organ-specific amyloidosis?

Answer 6

Alzheimer disease (deposition of amyloid-β protein)
Islet amyloid polypeptide (deposition of amylin in pancreatic islets...seen in DMII)

Question 7

How does amyloidosis appear with Congo stain under polarized light?

Answer 7

Apple green birefringence

Question 8

This is not an amyloidosis question. What is lipofuscin?

Answer 8

Yellow-brown pigment associated with normal aging

Might come up on a histology picture