A/13

Question 1

Cause of amyloidosis

Answer 1

inherited, inflammatory, neoplastic diseases cause aggregation of misfolded proteins

Question 2

What is the stain used to see amyloidosis?

Answer 2

congo red staining : birefringence

Question 3

Where do the amyloids deposit?

Answer 3

in the interstitial spaces

Question 4

How do the proteins aggregate to form amyloids?

Answer 4

When they misfold they expose more beta-sheets. These sheets easily stack to form crossed beta plates

Question 5

What are the 4 types of amyloid?

Answer 5

• amyloid light chain (AL) : primary
• amyloid associated (AA) : secondary
• amyloid beta (Ab)
• transthyretin (ATTR)

Question 6

Which amyloid type is localized and not systemic?

Answer 6

amyloid beta

Question 7

What is AL made of?

Answer 7

Misfolded proteins made of Ig light chains or fragments of it, produced by neoplastic plasma cells

Question 8

What an be the cause of AL?

Answer 8

Myeloma (cancer)

Question 9

What is AA made of?

Answer 9

Aggregate of SAA proteins (serum amyloid A)

Question 10

When is SAA produced and where?

Answer 10

In the liver in increased inflammatory states

Question 11

What is SAA associated with usually?

Answer 11

long standing inflammation (TB, autoimmune disorders)

Question 12

When can we see Ab?

Answer 12

In cerebral lesion of alzheimer’s disease

Question 13

What is Ab made of?

Answer 13

from APP (amyloid precursor protein)

Question 14

What is ATTR made of?

Answer 14

TTR protein : transthyretin

Question 15

When do we see ATTR?

Answer 15

In senile systemic amyloidosis

Question 16

Morphology of amyloidosis?

Answer 16

• organomegaly
• pressure atrophy of cells
• brown color on surface using iodine and sulphuric acid

Question 17

Organs most affected by amyloidosis (5)

Answer 17

• kidney
• spleen
• liver
• heart
• GI (gingiva)

Question 18

Where does amyloid deposit in kidney? (4)

Answer 18

glomeruli, mesangium peritubular interstitium, walls of vessels

Question 19

Amyloid patterns in spleen (2)

Answer 19

• sago spleen : in follicles

- in sinuses and pulp

Question 20

Morphology of liver affected by amyloid

Answer 20

• massively enlarged

- pale, waxy, grayish surface

Question 21

Where does amyloid spread in liver?

Answer 21

dissl spaces, surrounds parenchyma cells

Question 22

Morphology of amyloid in heart (2)

Answer 22

• dew-drop like subendocardial elevation

- deposit between myofibers (atrophy)

Question 23

Non specific clinical manifestations of amyloid

Answer 23

weakness, fatigue, weightloss

Question 24

Renal manifestation of amyloid

Answer 24

proteinuria, renal failure

Question 25

Cardiac manifestation of amyloid

Answer 25

restrictive cardiomyopathy