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Flashcards in amyloidosis + other Deck (59)
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1

amyloidosis is the

abnormal aggregation of protein (or their fragments into β-sheat

2

amyloidosis --> .... (it causes in the cellular level)

damage and apoptosis

3

amyloidosis - common types

1. AL (primary) 2. AA (secondary) 3. dialysis-related
4. heritable 5. age related (senile) systemic
6. organ specific

4

primary (AL) amyloidosis - mechanism

deposition of proteins from Ig Light chains (amyloid Light: AL)

5

primary (AL) amyloidosis is associated with

plasma cell dyscrasias (multiple myeloma)

6

primary (AL) amyloidosis often affects ...(including, and causes)

multiple organs including renal (nephrotic syndrome), cardiac (restrictive cardiomyopathy, arrhythmia), hematologic, (easy bruising, splenomegaly), GI (hepatomegaly), neurologic (neuropathy)

7

secondary (AA) amyloidosis - seen with

chronic inflammatory conditions such as RA, IBD, spondyloarthropathy, protracted infection, famlilial Mediterranean fever

8

secondary (AA) amyloidosis - fibrils composed of

serum Amyloid A

9

secondary (AA) amyloidosis - affects

often multisystem like AL amyloidosis

10

dialysis related amylodisis - fibrils composed of

β2-microglobulin

11

dialysis-related amylodisis - seen in

patients with end-stage-renal-disease and/or long-term dialysis

12

dialysis-related amylodisis may present as

Carpal tunnel syndrome

13

heritable amyloidosis (including....)?

heterogenous group of disorders, including familial amyloid polyneuropathies due to transthyretin gene mutation

14

heritable amyloidosis is a heterogenous group of disorders, including (and due to..)

familial amyloid polyneuropathies due to transthyretin gene mutation

15

familial amyloid polyneuropathies are due to

transthyretin gene mutation

16

age-related (senile) systemic is due to

deposition of normal (wild-type) transthyretin in myocardium and other sites

17

age-related (senile) systemic vs primary amyloidosis according to cardiac function

cardiac dysfunction in age-related (senile) systemic has a slower progression relative to primary amyloidosis

18

organ specific amyloidosis?

amyloid deposition localized to a single organ

19

organ specific amyloidosis - Most important form is

Alzheimer disease

20

Alzheimer disease - mechanism of amyloidosis

deposition of β-amyloid protein cleaved from amyloid precirsor protein (APP)

21

islet amyloid polypeptid is commonly seen in .... (and is caused by)

diabetes mellitus type 2
deposition of amylin in pancreatic islets

22

Amylin is a

peptide hormone that is cosecreted with insulin from the pancreatic beta-cell

23

amyloidosis - appearance

- congo red stain
- congo red stain shows apple green birefringence under polarized light
- H% eosin

24

β2-microglobulin fibrils - amyloidosis - type?

dialysis-related amyloidosis

25

islet amyloid polypeptid?

deposition of amylin in pancreatic islets (DM2)

26

Isolated atrial amyloidosis - due to

Organ specific amyloidosis --> due to ANP (COMMON IN NORMAL AGING)

27

p53 - product

Transcription factor for p21--> blocks G1 to S phase

28

p16 - product / associated tumor

Cyclin-dependent kinase inhibitor 2A
melanoma

29

NF1 / NF2- product

Ras GTPase activating protein (neurofibromin)
Merlin (schwannomin protein)

30

PTEN - associated tumor

1. breast
2. prostate
3. endometrial