Amyloidosis

Question 1

Amyloid

Answer 1

1) Mis-folded protein
2) Deposits in the extracellular space
3) Causing damage of tissues

Question 2

Amyloidosis

Answer 2

Abnormal aggregation of proteins (or their fragments)

Question 3

Amyloidosis aggregates into

Answer 3

β-pleated linear sheets —> insoluble fibrils

Question 4

Amyloidosis cause? and Deposition can be?

Answer 4

Cellular damage and apoptosis

Deposition can be systemic or localized

Question 5

Amyloid deposits visualized by

Answer 5

Congo red stain

Question 6

non-polarized light

Answer 6

Red/orange

Question 7

polarized light

Answer 7

apple-green birefringence

Question 8

Systemic Amyloidosis

Answer 8

Amyloid deposition in multiple organs

Question 9

Primary amyloidosis

Answer 9

1) Systemic deposition of AL amyloid
2) Derived from immunoglobulin light chain
3) Associated with plasma cell dyscrasias (multiple myeloma

Question 10

Secondary amyloidosis

Answer 10

Systemic deposition of AA amyloid
Derived from serum amyloid-associated protein (SAA) 
SAA is an acute phase reactant that is increased in:
Chronic inflammatory states:
- Rheumatoid arthritis 
- inflammatory bowel disease 
Malignancy 
Familial Mediterranean fever (FMF)

Question 11

most common organ involved in amyloidosis

Answer 11

Kidney

Question 12

Classic findings include

Answer 12

Nephrotic Syndrome (kidney: most common organ involved) 
Restrictive cardiomyopathy or arrythmia
Tongue enlargement
Malabsorption
Hepatosplenomegaly
Easy bruising
Neuropathy
Carpal tunnel syndrome

Question 13

Diagnosis requires tissue biopsy from?

Answer 13

abdominal fat and rectum

Question 14

Amyloid can be removed (true or false)

Answer 14

False

Question 15

Damaged organs must be transplanted (true or false)

Answer 15

True

Question 16

Localized amyloidosis

Answer 16

Amyloid deposition usually localized to a single organ

Question 17

Types of localized amyloidosis:

Answer 17

Senile cardiac amyloidosis
Familial amyloid cardiomyopathy 
Non-insulin-dependent diabetes mellitus (type II)
Alzheimer disease
Dialysis associated amyloidoses
Medullary carcinoma of the thyroid 
Familial amyloid neuropathy

Question 18

Senile cardiac amyloidosis

Answer 18

Non mutated serum transthyretin

Deposits in the heart

Question 19

Familial amyloid cardiomyopathy

Answer 19

Mutated serum transthyretin
Deposits in the heart
Leading to restrictive cardiomyopathy
Hereditary
5% of African Americans are carriers of mutant allele

Question 20

Non-insulin-dependent diabetes mellitus (type II):

Answer 20

Amylin (derived from insulin)

Deposits in the islets of the pancreas

Question 21

Alzheimer disease

Answer 21

Aβ amyloid (derived from β-amyloid precursor protein)

Deposits in the brain forming amyloid plaques

Question 22

Dialysis associated amyloidosis

Answer 22

β2-microglobulin

Deposits in joints

Question 23

Medullary carcinoma of the thyroid

Answer 23

Calcitonin (produced by tumor cells)

Deposits within the tumor

Question 24

Familial amyloid neuropathy

Answer 24

Mutated transthyretin