Amyloidosis

Question 1

What is the structure of amyloid deposits?

Answer 1

A ß-pleated structure of amyloid with another substance acting as a linking element

Question 2

What stain is used for amyloidosis? How does it work? What is the characteristic pattern?

Answer 2

Congo Red
Intercalates into ß-pleated sheet, dispersing light
Apple Green Birefringence

Question 3

Describe where primary amyloidosis is usually seen, what the depositing molecule is, what the amyloid structure is, and where symptoms are found

Answer 3

• B cell dyscrasia (Multiple Myeloma)
• Immunoglobulin lambda light chains
• AL Structure
• Skin, GI, Heart, Tongue

Remember - LIGHT (Light chains, Integument, GI, Heart, Tongue)

Question 4

Describe where secondary amyloidosis is usually seen, what the depositing molecule is, what the amyloid structure is, and where symptoms are found

Answer 4

• Chronic Inflammation (TB, rheumatoid arthritis)
• Serum Amyloid Associated Protein (SAA)
• AA structure (remember autoimmune for AA)
• Deposits in abdominal organs - kidney (nephrotic syndrom), liver (portal hypertension and liver failure), spleen (follicles -> sagosplenic follicles, red pulp -> lardaceous spleen)

Question 5

Describe where senile cerebral amyloidosis is usually seen, what the depositing molecule is, what the amyloid structure is.

Answer 5

• Alzheimer’s disease
• APP Precurosr
• A-Beta structure

Question 6

Describe where Familial Mediterranean Fever is usually seen, what the depositing molecule is, what the amyloid structure is.

Answer 6

• Hereditary
• SA Precursor Protein
• AA Amyloid structure

Question 7

Describe where amyloidotic neuropathies is usually seen, what the depositing molecule is, what the amyloid structure is.

Answer 7

• Hereditary
• Transthyretin protein
• ATTR amyloid structure

Question 8

Describe where sendocrine amyloidosis is usually seen, what the depositing molecule is, what the amyloid structure is.

Answer 8

• Causes Medullary Thyroid Carcinoma (MTC)
• Calcitonin precursor
• ACal amyloid

Question 9

Describe where hemodialysis-associated amyloidosis is usually seen, what the depositing molecule is, what the amyloid structure is.

Answer 9

• Chronic renal failure patients
• ß2-microglobulin
• Aß2m amyloid structure