Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Pathology 1 Block 2 > Amyloidosis > Flashcards

Amyloidosis Flashcards

1
Q

what is amyloidosis?

A

misfolded proteins that deposit in Extracellular space of tissues and organs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what is the composition of amyloid fibrils?

A

continuous nonbranching fibrils with cross β-pleated sheet conformation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what is the stain used to view amyloid proteins?

A

congo red

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what are the 3 forms of amyloid fibrils?

A
  1. AL (amyloid light chain)
  2. AA (amyloid associated)
  3. Aβ amyloid
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

from where is AL (amyloid light chain) derived from?

where can we find AL amyloid proteins?

A

from Ig light chains produced by plasma cells

plasma cell tumors (contains complete Ig light chain: ʎ and ƙ)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

from where does AA (amyloid associated) come from?

when is it seen?

A

from an unique non-Ig protein (SAA) synthesized by the liver

seen in inflammation due to release of SAA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

from where does Aβ amyloid come from?

A

produced by the β-amyloid precursor protein (Alzheimer disease CNS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what are another 3 types of amyloidosis besides the 3 common types?

A
  1. Amyloid Transthyretin (ATTR)
  2. β2-microglobulin
  3. Prion protein
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what is normal transthyretin? where is it found normally?

A
  • normal serum protein that binds and transport thyroxine and retinol
  • hearts of aged individuals
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what is amyloid transthyretin?

A

mutant form of transthyretin that is found deposisted in a group of genetic diseases: familial amyloid polyneuropathies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what is ß2-microglobulin?

why is it bad if it accumulates?

A
  • component of MHC class 1 molecules
  • complicate the course of patients on long term hemodialysis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what is Prion protein?

A
  • misfolded ‘prion’ protein
  • clump in extracellular space
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is the Amyloidosis Pathogenesis?

A
  • proteosomes or extracellular macrophages do not degrade amyloid proteins
  • they accumulate
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what are the 4 classifications for amyloidosis?

A
  1. Systemic Amyloidosi
  2. Hereditary Amyloidosis
  3. Localized Amyloidosis
  4. Endocrine Amyloidosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what diseases can we find in the Systemic Amyloidosis category?

A
  1. immune dyscrasias
  2. Reactive systemic amyloidosis
  3. hemolysis associated amyloidosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what diseases can we find in the Hereditary Amyloidosis category?

A
  1. Familial Mediterranean fever
  2. Familial amyloidotic neuropathies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what diseases can we find in theLocalized Amyloidosis category?

A
  1. Alzheimer
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what diseases can we find in the Endocrine Amyloidosis category?

A
  1. Medullary carcinoma thyroid
  2. Diabetes Mellitus (Type II)​
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what is immune dyscrasias?

A

its AL type amyloid with systemic distribution

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what is the most common type of immune dyscrasias (systemic amyloidosis)?

what do you see in a lab report to detect this?

what is immune dyscrasias?

A
  • multiple myeloma
  • increased ESR
  • plasma cell malignancy
21
Q

what is being produced in abnormally large quantities in immune dyscrasias?

A

produces abnormally large quantity of ‘M’ protein

22
Q

how are light chains that may escape in urine referred to?

A

“Bence-Jones” protein

23
Q

how is reactive systemic amyloidosis referred to?

A

“secondary amyloidosis”

24
Q

reactive systemic amyloidosis has what type of amyloid protein?

25
reactive systemic amyloidosis can be more frequently seen in what patients?
heroin abusers who inject drugs subcutaneously
26
what diseases can have reactive systemic amyloidosis? what other disease have it?
non-immunocyte derived tumors: Renal cell carcinoma, Hodgkin lymphoma Tuberculosis, Bronchiectasis, osteomyelitis
27
what disease is this? what is happening in the picture?
reactive systemic amyloidosis ## Footnote Lung: note dense pink material within wall of airways due to chronic inflammation.
28
what is hemodialysis-associated amyloidosis?
ß2-microglobulin deposition
29
in what patients do we find ß2 microglobulin deposited?
those with renal failure
30
why is there ß2 microglobulin deposition?
due to failure of filtration through hemodialysis membrane
31
ß2 microglobulin deposition patients will present with what ?
carpal tunnel syndrome
32
what type of amyloidosis is this?
Hemodialysis-associated amyloidosis
33
what is Familial Mediterranean fever?
‘autoinflammatory’ condition, due to abnormally high production of IL-1, in response to inflammatory stimuli
34
what characterizes Hereditary Amyloidosis?
fevers Inflammation of serosal surfaces (pleura, peritoneum, synovium)
35
what is Familial amyloidotic polyneuropathies? what is the amyloid protein found?
deposition of amyloid in peripheral and autonomic nerves ATTR (transthyretin)
36
what type of amyloidosis protein is found in Familial Mediterranean fever?
AA type, derived from SAA
37
what is Familial amyloid cardiomyopathy?
mutant ATTR deposition that presents as restrictive cardiomyopathy
38
What is an example of localized amyloidosis? what is it?
* Alzheimer * deposition of Aβ amyloid derived from APP
39
what is this an example of? what disease is this?
localized amyloidosis ## Footnote Amyloid angiopathy in Alzheimer disease: Congo red positive
40
what is this?
Medullary carcinoma thyroid: note presence of amyloid in this malignancy. Note thyroid follicles containing pink colloid.
41
what is an example of endocrine amyloidosis?
Medullary carcinoma of thyroid found in MEN syndrome
42
what is this?
Medullary thyroid carcinoma: Amyloid (arrow) is often associated with these tumors. Medullary thyroid carcinoma can be sporadic, but also has familial forms, as well as being a component of multiple endocrine neoplasia, type 2 (MEN 2).
43
what is this?
Diabetes mellitus, amyloidosis of the islets * The single islet in the center of the image contains a prominent amount of acellular eosinophilic material (amyloid).
44
In amyloidosis, affected organs tend to become what? how will they look?
enlarged with waxy appearance
45
In amyloidosis of the spleen, the deposits are usually limited to what 2 regions?
1. Deposits limited to **splenic follicles**, with tapioca like granules: **“sago spleen”** 2. wall of the **splenic** **sinuses**, and **connective** **tissue** framework of **red** **pulp**: “**lardaceous** **spleen**”
46
amyloidosis of the heart usually present with what?
restrictive cardiomyopathy
47
what is this? what is presented in this picture?
* Restrictive cardiomyopathy or heart amyloidosis. * The heart is firm and rubbery with a waxy cut surface. The atria are markedly dilated and the left atrial endocardium, normally smooth, has yellow-brown amyloid deposits that give texture to the surface.
48
what is this?
amyloidosis of the tongue
49
what is this?
apple green birefringence noted on polarizing microscopy

Pathology 1 Block 2 (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions