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Pathoma LAH > Basics: 1.5: Amyloid > Flashcards

Basics: 1.5: Amyloid Flashcards

1
Q

What do thyroid tumor cells in an amyloid background indicate?

A

medullary carcinoma of the thyroid

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2
Q

This is systemic deposition of AL amyloid derived from the Ig light chain; assoc with plasma cell dyscrasias.

A

1a amyloidosis

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3
Q

What is amylin?

A

an amyloid that is a byproduct of insulin that can deposit in the islets of the pancreas

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4
Q

What is senile cardiac amyloidosis?

A
  • non-mutated serum transthyretin deposits in the heart
  • present in 25% of individuals >80yo
  • usually asymptomatic
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5
Q

What is 1a amyloidosis?

A
  • systemic deposition of AL amyloid
    • derived from the Ig light chain
    • assoc with plasma cell dyscrasias
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6
Q

What is systemic deposition of AA amyloid (derived from SAA) called?

A

2a amyloidosis

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7
Q

What are non-mutated serum transthyretin deposits in the heart called?

A

senile cardiac amyloidosis

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8
Q

dx?

  • mutated serum transthyretin
  • deposits in the heart
  • leads to restrictive cardiomyopathy
  • 5% of African Americans are carriers
A

familial amyloid cardiomyopathy

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9
Q

Name the localized amyloidoses.

A
  1. familial amyloid cardiomyopathy
  2. senile cardiac amyloidosis
  3. DM associated
  4. Alzheimers
  5. dialysis-associated amyloidosis
  6. medullary carcinoma of the thyroid
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10
Q

What is dialysis-associated amyloidosis?

A
  • beta-2-microglobulin deposits in joints
    • in dialysis pts
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11
Q

What is medullary carcinoma of the thyroid?

A

a thyroid tumor derived from C cells, leading to overproduction of calcitonin and then amyloid deposition in the thyroid

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12
Q

Dx?

  • beta-2-microglobulin deposits in joints
A

dialysis-associated amyloidosis

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13
Q

How does amyloid look under the microscope?

A

congo red staining with apple green birefringence

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14
Q

What is the usual configuration of amyloid-damaged tissue?

A

Beta-pleated sheets

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15
Q

How can amyloidosis occur in Alzheimers disease?

A
  • beta-amyloid precursor protein on chromosome 21 deposits into the brain as ABeta
    • forms amyloid plaques
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16
Q

Systemic amyloidosis is divided into ____ and ____ types.

A
  • 1a
  • 2a
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17
Q

How can amyloidosis occur in DM?

A

type 2 DM: too much insulin = insulin byproduct produced (amylin)amylin deposits into islets of the pancreas

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18
Q

What are the features of Familial Mediterranean Fever?

A

dysfunction of the neutrophils causing activation w/o infectionauto recessive inheritance in persons of Mediterranean origin fever; serosal surfaces inflamed; tongue enlargement, hepatosplenomegaly, malabsorption

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19
Q

How is amyloidosis definitively diagnosed?

A

a tissue biopsy (usu a fat pad- abdominal or rectal)

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20
Q

What is the usual configuration of amyloid-damaged tissue?

A

Beta-pleated sheets

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21
Q

What is restrictive cardiomyopathy?

A
  • stiffened heart
  • can’t dilate as well or pump properly
  • leads to HF
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22
Q

What is 2a amyloidosis?

A

systemic deposition of AA amyloid (derived from SAA)

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23
Q

Where does amyloid most commonly deposit? What does this cause?

A
  • the kidney
  • nephrotic syndrome
24
Q

What is an amyloid?

A

a misfolded protein that deposits into the extracellular space –> tissue damage

25
Q

What is SAA?

A
  • an acute phase reactant increased in:
    • inflammation
    • malignancy
    • Familial Mediterranean fever
26
Q

What kind of amyloidosis category does senile cardiac amyloidosis fall into?

A

localized amyloidosis

27
Q

What is familial amyloid cardiomyopathy?

A
  • mutated serum transthyretin deposits in the heart
  • leads to restrictive cardiomyopathy
  • 5% of African Americans are carriers
28
Q

What is nephrotic syndrome?

A

a large loss of protein (>3g in 24 hours) in the urine

29
Q

How are the affected organs treated in amyloidosis?

A

no tx- they have to be transplanted

30
Q

Where does amyloid tend to deposit?

A

around BVs

31
Q

What is the 2nd most common protein in the blood?

A

serum transthyretin

32
Q

What is the difference btw familial amyloid cardiomyopathy and senile cardiac amyloidosis ?

A
  • familial = mutated serum transthyretin
  • senile = non-mutated serum transthyretin
33
Q

Why does dialysis-assoc amyloidosis occur?

A
  • Dialysis pts have increased amounts if Beta-2-microglobulin in the blood bc it isn’t filtered out properly
    • it then accumulates in joints
34
Q

What are the features of Familial Mediterranean Fever?

A
  • dysfunction of the neutrophils causing activation w/o infection
  • auto recessive inheritance in persons of Mediterranean origin
  • fever; serosal surfaces affected
35
Q

Dx?

  • dysfunction of the neutrophils causing activation w/o infection
  • auto recessive inheritance in persons of Mediterranean origin
  • fever; serosal surfaces affected
A

Familial Mediterranean Fever

36
Q

What is the amyloid that is a byproduct of insulin that can deposit in the islets of the pancreas?

A

amylin

37
Q

What is a misfolded protein that deposits into the extracellular space called?

A

an amyloid

38
Q

How is medullary carcinoma of the thyroid definitively diagnosed?

A

fine needle aspiration of the thyroid

39
Q

What is AL amyloid derived from?

A

Ig light chain

40
Q

Where does amyloid most commonly deposit? What does this cause?

A
  • the kidney
  • nephrotic syndrome
41
Q

2a amyloidosis can result from a _____, like autoimmune disease (SLE, RA, Crohn’s, etc), ______, or _____.

A
  • chronic inflammatory state
  • malignancy
  • Familial Mediterranean fever
42
Q

Why are amyloids bad?

A

they damage tissues

43
Q

What can happen to the heart in amyloidosis?

A
  • restrictive cardiomyopathy
  • arrhythmia
44
Q

Amyloid deposition can be _____ or _____.

A
  • systemic
  • localized
45
Q

What kind of amyloidosis is dialysis-associated?

A

localized

46
Q

2a amyloidosis can result from a _____, like autoimmune disease (SLE, RA, Crohn’s, etc), or _____.

A
  • chronic inflammatory state
  • malignancy
47
Q

What is AL amyloid derived from?

A

Ig light chain

48
Q

What is the link btw Alzheimers and Down Syndrome?

A
  • the beta-amyloid precursor protein is on chromosome 21 and DS = trisomy 21
    • early onset Alzheimers seen in DS individuals
49
Q

What is Beta-2-microglobulin?

A

a supportive protein for MHC-1 expression

50
Q

What is serum transthyretin?

A

the 2nd most common protein in the blood

51
Q

What is dialysis-associated amyloidosis?

A

beta-2-microglobulin deposits in joints

52
Q

What is AA amyloid?

A

an amyloid derived from SAA

53
Q

How is amyloidosis definitively diagnosed?

A

a tissue biopsy (usu a fat pad- abdominal or rectal)

54
Q

What is a large loss of protein (>3g in 24 hours) in the urine called?

A

nephrotic syndrome

55
Q

Why does dialysis-assoc amyloidosis occur?

A
  • Dialysis pts have increased amounts if Beta-2-microglobulin in the blood bc it isn’t filtered out properly
    • it then accumulates in joints
56
Q

Where is the beta-amyloid precursor protein gene located?

A

on chromosome 21

57
Q

What is localized amyloidosis?

A

amyloid deposition localized to a single organ or tissue

Pathoma LAH (77 decks)

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