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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

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2
Q

what is the composition of amyloid fibrils?

A

continuous nonbranching fibrils with cross β-pleated sheet conformation

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3
Q

what is the stain used to view amyloid proteins?

A

congo red

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4
Q

what are the 3 forms of amyloid fibrils?

A
  1. AL (amyloid light chain)
  2. AA (amyloid associated)
  3. Aβ amyloid
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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)

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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

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7
Q

from where does Aβ amyloid come from?

A

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

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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
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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
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10
Q

what is amyloid transthyretin?

A

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

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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
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12
Q

what is Prion protein?

A
  • misfolded ‘prion’ protein
  • clump in extracellular space
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13
Q

what is the Amyloidosis Pathogenesis?

A
  • proteosomes or extracellular macrophages do not degrade amyloid proteins
  • they accumulate
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14
Q

what are the 4 classifications for amyloidosis?

A
  1. Systemic Amyloidosi
  2. Hereditary Amyloidosis
  3. Localized Amyloidosis
  4. Endocrine Amyloidosis
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15
Q

what diseases can we find in the Systemic Amyloidosis category?

A
  1. immune dyscrasias
  2. Reactive systemic amyloidosis
  3. hemolysis associated amyloidosis
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16
Q

what diseases can we find in the Hereditary Amyloidosis category?

A
  1. Familial Mediterranean fever
  2. Familial amyloidotic neuropathies
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17
Q

what diseases can we find in theLocalized Amyloidosis category?

A
  1. Alzheimer
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18
Q

what diseases can we find in the Endocrine Amyloidosis category?

A
  1. Medullary carcinoma thyroid
  2. Diabetes Mellitus (Type II)​
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19
Q

what is immune dyscrasias?

A

its AL type amyloid with systemic distribution

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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?

A

AA type

25
Q

reactive systemic amyloidosis can be more frequently seen in what patients?

A

heroin abusers who inject drugs subcutaneously

26
Q

what diseases can have reactive systemic amyloidosis? what other disease have it?

A

non-immunocyte derived tumors: Renal cell carcinoma, Hodgkin lymphoma

Tuberculosis, Bronchiectasis, osteomyelitis

27
Q

what disease is this?

what is happening in the picture?

A

reactive systemic amyloidosis

Lung: note dense pink material within wall of airways due to chronic inflammation.

28
Q

what is hemodialysis-associated amyloidosis?

A

ß2-microglobulin deposition

29
Q

in what patients do we find ß2 microglobulin deposited?

A

those with renal failure

30
Q

why is there ß2 microglobulin deposition?

A

due to failure of filtration through hemodialysis membrane

31
Q

ß2 microglobulin deposition patients will present with what ?

A

carpal tunnel syndrome

32
Q

what type of amyloidosis is this?

A

Hemodialysis-associated amyloidosis

33
Q

what is Familial Mediterranean fever?

A

‘autoinflammatory’ condition, due to abnormally high production of IL-1, in response to inflammatory stimuli

34
Q

what characterizes Hereditary Amyloidosis?

A

fevers

Inflammation of serosal surfaces (pleura, peritoneum, synovium)

35
Q

what is Familial amyloidotic polyneuropathies?

what is the amyloid protein found?

A

deposition of amyloid in peripheral and autonomic nerves

ATTR (transthyretin)

36
Q

what type of amyloidosis protein is found in Familial Mediterranean fever?

A

AA type, derived from SAA

37
Q

what is Familial amyloid cardiomyopathy?

A

mutant ATTR deposition that presents as restrictive cardiomyopathy

38
Q

What is an example of localized amyloidosis?

what is it?

A
  • Alzheimer
  • deposition of Aβ amyloid derived from APP
39
Q

what is this an example of?

what disease is this?

A

localized amyloidosis

Amyloid angiopathy in Alzheimer disease: Congo red positive

40
Q

what is this?

A

Medullary carcinoma thyroid: note presence of amyloid in this malignancy. Note thyroid follicles containing pink colloid.

41
Q

what is an example of endocrine amyloidosis?

A

Medullary carcinoma of thyroid found in MEN syndrome

42
Q

what is this?

A

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
Q

what is this?

A

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
Q

In amyloidosis, affected organs tend to become what?

how will they look?

A

enlarged with waxy appearance

45
Q

In amyloidosis of the spleen, the deposits are usually limited to what 2 regions?

A
  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
Q

amyloidosis of the heart usually present with what?

A

restrictive cardiomyopathy

47
Q

what is this?

what is presented in this picture?

A
  • 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
Q

what is this?

A

amyloidosis of the tongue

49
Q

what is this?

A

apple green birefringence noted on polarizing microscopy

Pathology 1 Block 2 (8 decks)

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