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CVPR exam 2 > Pathoma- Amyloid > Flashcards

Pathoma- 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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2
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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3
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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4
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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4
Q

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

A

2a amyloidosis

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4
Q

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

A

senile cardiac amyloidosis

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4
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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4
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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5
Q

What is dialysis-associated amyloidosis?

A

beta-2-microglobulin deposits in joints dialysis pts

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5
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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6
Q

Dx? beta-2-microglobulin deposits in joints

A

dialysis-associated amyloidosis

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7
Q

How does amyloid look under the microscope?

A

congo red staining with apple green birefringence

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9
Q

What is the usual configuration of amyloid-damaged tissue?

A

Beta-pleated sheets

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10
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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11
Q

Systemic amyloidosis is divided into ____ and ____ types.

A

1a; 2a

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11
Q

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

A

senile cardiac amyloidosis

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11
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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12
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 inflamed; tongue enlargement, hepatosplenomegaly, malabsorption

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12
Q

How is amyloidosis definitively diagnosed?

A

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

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14
Q

What is the usual configuration of amyloid-damaged tissue?

A

Beta-pleated sheets

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14
Q

Name the localized amyloidoses.

A
  1. familial amyloid cardiomyopathy 2. senile cardiac amyloidosis 3. DM associated 4. Alzheimers 5. dialysis-associated amyloidosis
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15
Q

What is restrictive cardiomyopathy?

A

stiffened heart can’t dilate as well or pump properly, leading to HF

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16
Q

How can amyloidosis occur in Alzheimers disease?

A

beta-amyloid precursor protein on chromosome 21 forms ABeta amyloid forms amyloid plaques in the brain

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18
Q

What is 2a amyloidosis?

A

systemic deposition of AA amyloid derived from SAA

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19
Q

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

A

the kidney; nephrotic syndrome

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20
Q

What is an amyloid?

A

a misfolded protein that deposits into the extracellular space

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20
Q

What is SAA?

A

an acute phase reactant increased in inflammation, malignancy, and Familial Mediterranean fever

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20
Q

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

A

localized amyloidosis

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21
Q

What is familial amyloid cardiomyopathy?

A

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

22
Q

What is nephrotic syndrome?

A

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

23
Q

How are the affected organs treated in amyloidosis?

A

no tx- they have to be transplanted

24
Q

Where does amyloid tend to deposit?

A

around BVs

25
Q

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

A

serum transthyretin

27
Q

What is AA amyloid?

A

an amyloid derived from SAA

28
Q

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

A

familial = mutated serum transthyretin senile = non-mutated serum transthyretin

28
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

29
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

31
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

31
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 Alzheimers seen in DS individuals

33
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 inflamed

34
Q

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

A

amylin

36
Q

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

A

an amyloid

37
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

What is restrictive cardiomyopathy?

A

stiffened heart can’t dilate as well or pump properly, leading to HF

43
Q

Systemic amyloidosis is divided into ____ and ____ types.

A

1a; 2a

44
Q

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

A

familial = mutated serum transthyretin senile = non-mutated serum transthyretin

46
Q

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

A

chronic inflammatory state; malignancy; Familial Mediterranean fever

48
Q

Why are amyloids bad?

A

they damage tissues

50
Q

What can happen to the heart in amyloidosis?

A

restrictive cardiomyopathy arrhythmia

51
Q

Where does amyloid tend to deposit?

A

around BVs

53
Q

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

A

the kidney; nephrotic syndrome

54
Q

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

A

senile cardiac amyloidosis

56
Q

Amyloid deposition can be _____ or _____.

A

systemic; localized

57
Q

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

A

familial = mutated serum transthyretin senile = non-mutated serum transthyretin

58
Q

How are the affected organs treated in amyloidosis?

A

no tx- they have to be transplanted

59
Q

What kind of amyloidosis is dialysis-associated?

A

localized

60
Q

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

A

chronic inflammatory state; malignancy

61
Q

What is AL amyloid derived from?

A

Ig light chain

62
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

63
Q

What is Beta-2-microglobulin?

A

a supportive protein for MHCI expression

64
Q

What is serum transthyretin?

A

the 2nd most common protein in the blood

65
Q

What is dialysis-associated amyloidosis?

A

beta-2-microglobulin deposits in joints

66
Q

What is AA amyloid?

A

an amyloid derived from SAA

67
Q

How is amyloidosis definitively diagnosed?

A

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

68
Q

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

A

nephrotic syndrome

69
Q

What is medullary carcinoma of the thyroid?

A

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

70
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

71
Q

Where is the beta-amyloid precursor protein gene located?

A

on chromosome 21

72
Q

What can happen to the heart in amyloidosis?

A

restrictive cardiomyopathy arrhythmia

73
Q

What is localized amyloidosis?

A

amyloid deposition localized to a single organ or tissue

74
Q

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

A

senile cardiac amyloidosis

CVPR exam 2 (29 decks)

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