3. amyloid

Question 1

Amyloid

Answer 1

Amyloid is a misfolded protein that deposits in the extracellular space that injure tissue

Question 2

Physical Nature of Amyloid

Answer 2

a cross-β-pleated sheet conformation

Question 3

Amyloidoma

Answer 3

Amyloidoma (tumoral amyloidosis) = solitary localized tumorlike deposit

Question 4

staining in macroscope

Answer 4

Painting cut-surface with iodine imparts a brown- yellow colour, which changes to bluish-violet after application of sulphuric acid

Question 5

type of stain plus how it appears in microscope

Answer 5

Congo red- on light microscope (pink or red colour) and on polarized microscope (apple green birefringence)
eosinophillic

Question 6

Clinical Manifestation

Answer 6

usually asymptomatic
non specific weakness, loss of weight and light-headedness

Question 7

classification of amyloidosis

Answer 7

systemic
localized
hereditary

Question 8

systemic amyloidosis

Answer 8

kidney most common

Question 9

systemic classiefied into

Answer 9

primary and secondary

Question 10

primary amyloidosis

Answer 10

deposistion of AI amyloids derived from light chain ig
not secondary to other dieseases

Question 11

secondary amyloidosis

Answer 11

Secondary to inflammatory conditions
AA (amyloid-associated) protein derived from precursor SAA (serum amyloid-associated) protein
inflammation causes SAA to change to AA then AA deposits

Question 12

amyloidosis in kidney causes

Answer 12

Nephrotic syndrome and renal failure

Question 13

amyloid deposition in spleen causes the 2 types

Answer 13

1. Sago spleen: deposits largely limited to splenic follicles leading to Tapioca-like granules (in white pulp)
2. Lardaceous spleen: involvement of walls of splenic sinuses and connective tissue framework of (red pulp), leading to large map like areas

Question 14

amyloid in liver deposits in

Answer 14

space of disse
causes pressure atrophy
then hepatomegaly?

Question 15

cardiac amyloidosis

Answer 15

causes Restrictive cardiomyopathy, arrhythmia, congestive cardiac failure and constrictive pericarditis

Question 16

types of amyloidosis in cardiac amyloidosis
3

Answer 16

Senile cardiac amyloidosis-Non mutated TTR (wt-TTR)- wild type

Familial amyloid cardiomyopathy -Mutated TTR (m-TTR)-hereditary transthyretin amyloidosis (ATTRm)

AL amyloid

Question 17

Dialysis associated amyloidosis

Answer 17

in dialysis the B2M not filtered well
its found in platlets and nucleated cells helps the MHC to be expressed well
B2M accumulates in joints causing joint paints for example
carpal tunnel syndrome

Question 18

localized amyloidosis

Answer 18

Often caused by local production of immunoglobulin light chains (Localized AL)

Question 19

Senile cerebral amyloidosis (alzheimers)

Answer 19

deposition of Ab amyloid which is derived from B amyloid precursor which is coded by chromosome 21 so down syndrome patients will develop alzheimers early

Question 20

Cerebral amyloid angiopathy (CAA)

Answer 20

Deposition of amyloid fibrils within the walls of blood vessels of the brain and meninges

Question 21

type 2 DM amyloid

Answer 21

amylin which is a byproduct of insulin when excess in DM2 , deposits in the islets of pancreas

Question 22

medullary thyroid carcinoma

Answer 22

tumour in c cells - more calcitonin - calcitonin deposits in the gland