Amyloidosis

Question 1

What is Amyloid

Answer 1

Pathological proteinaceous substance deposited in the extra cellular space
Systemic disease abnormal protein folding

Question 2

How is the differentiation of Amyloids

Answer 2

Congo red stain

Question 3

How many biochemical forms does Amyloid have?

Answer 3

3 major
Several minor

Question 4

How it he physiologic nature of the amyloid

Answer 4

Continuous non branching fibrils

Question 5

How is the chemical nature of the amyloid

Answer 5

Fibril proteins
P component
Glycoproteins

Question 6

Classification of amyloidosis

Answer 6

Primary= AL
Reactive Systemi (Secondary)= AA

Question 7

What is AL

Answer 7

Amyloid Light Chain
Derived from Ig light chains
Produced in plasma cells
Primary

Question 8

AL derived from

Answer 8

Ig light chains produced in plasma cells

Question 9

Which Amyloid type is primary

Answer 9

AL

Question 10

Abeta amyloid produced from

Answer 10

Beta amyloid precursor protein

Question 11

ABETA amyloid found in

Answer 11

The cerebral lesions of Alzheimer’s disease

Question 12

Which amyloid is the secondary

Answer 12

AA

Question 13

Which amyloid does not have structural homology to Igs

Answer 13

AA

Question 14

AA fibrils derived by

Answer 14

Proteolysis from a precursor in the serum called Serum Amyloid Associated which is synthesized in the liver and circulates in association with Heavy Dense Lipoprotein

Question 15

SAA is increased in

Answer 15

Inflammatory states as part of the acute phase response so it is associated with chronic inflammation

Question 16

What is Transthyretinin protein

Answer 16

Anormal protein
Binds and transports thyroxine and retinol
Have mutations

Question 17

What is Senile Systemic Amyloidosis

Answer 17

Caused by accumulation of Transthyretin
Affects heart and tendons of elderly people

Question 18

Beta microglobulin

Answer 18

Component of MHC class 1 molecules
Accumulates in long term hemodialysis

Question 19

Primary and secondary amyloids are associated with which diseases

Answer 19

Primary = ımmunocyte disorder, plasma cell dyscraia , myeloma
Secondary= complication of an underlying chronic inflammatory or tissue destructive process

Question 20

Reactive systemic amyloidosis

Answer 20

Amyloid deposits in this pattern are systemic distribution and are composed of AA protein associated with inflammatory conditions as TBC,bronchiectasis,chronic osteomyelitis or CT disorders, inflammatory bowel disease

Question 21

Hemodialysis associated amyloidosis

Answer 21

Heredofamilial amyloidosis
Endocrine amyloid
Amyloid of aging

Question 22

Which organ amyloidosis is the most common and potentially the most serious form of organ involvement

Answer 22

Kidney

Question 23

2 forms of Spleen deposits

Answer 23

1- Sago Spleen : splenic follicles producing tapioca like granules
2- Lardeceous Spleen : the walls of the spleen sinuses and CT framework

Question 24

Accumulation of Liver

Answer 24

First: Disse spaces
Then: hepatic parenchyma cells
Sinusoids causes pressure atrophy