Amyloidosis

Question 1

What is amyloidosis?

Answer 1

Misfolded protein(s) that deposits in the extracellular space, thereby damaging tissues

Question 2

Features of amyloidosis

Answer 2

• Beta-pleated sheet configuration

- Congo red staining; apple-green birefringence when viewed microscopically under polarized light

Question 3

What is primary amyloidosis?

Answer 3

Systemic deposition of AL amyloid, which is derived from immunoglobulin light chain. Associated with plasma cell dycrasias (multiple myeloma - overproduction of light chains)

Question 4

What is secondary amyloidosis?

Answer 4

Systemic deposition of AA amyloid served from SAA which is an acute phase reactant that is increased in chronic inflammatory states, malignancy, and Familial Mediterranean fever (FMF)

Question 5

Classic clinical findings of Systemic Amyloidosis

Answer 5

1) Nephrotic Syndrome - kidney is the most common organ involved
2) Restrictive cardiomyopathy or ayyhythmia
3) Tongue enlargement, malabsorption, and hepatosplenomegaly

Question 6

How do you diagnose Amyloidosis?

Answer 6

Tissue biopsy. Abdominal fat pad and rectum are the most easily accessible biopsy targets

Question 7

Treatment of Amyloidosis?

Answer 7

Transplant

Question 8

Examples of Localized Amyloidosis

Answer 8

1) Senile Cardiac amyloidosis
2) Familial amyloid cardiomyopathy
3) Non-insulin-dependent diabetes mellitus
4) Alzheimer diseae
5) Dialysis-associated amyloidosis
6) Medullary carcinoma of the thyroid