CVPR exam 2 > Pathoma- Amyloid > Flashcards

Pathoma- Amyloid Flashcards

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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
What is 2a amyloidosis?
systemic deposition of AA amyloid derived from SAA
19
Where does amyloid most commonly deposit? What does this cause?
the kidney; nephrotic syndrome
20
What is an amyloid?
a misfolded protein that deposits into the extracellular space
20
What is SAA?
an acute phase reactant increased in inflammation, malignancy, and Familial Mediterranean fever
20
What kind of amyloidosis category does senile cardiac amyloidosis fall into?
localized amyloidosis
21
What is familial amyloid cardiomyopathy?
mutated serum transthyretin deposits in the heart leads to restrictive cardiomyopathy 5% of African Americans are carriers
22
What is nephrotic syndrome?
a large loss of protein (\>3g in 24 hours) in the urine
23
How are the affected organs treated in amyloidosis?
no tx- they have to be transplanted
24
Where does amyloid tend to deposit?
around BVs
25
What is the 2nd most common protein in the blood?
serum transthyretin
27
What is AA amyloid?
an amyloid derived from SAA
28
What is the difference btw familial amyloid cardiomyopathy and senile cardiac amyloidosis ?
familial = mutated serum transthyretin senile = non-mutated serum transthyretin
28
Why does dialysis-assoc amyloidosis occur?
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
What are the features of Familial Mediterranean Fever?
dysfunction of the neutrophils causing activation w/o infection auto recessive inheritance in persons of Mediterranean origin fever; serosal surfaces affected
31
Dx? dysfunction of the neutrophils causing activation w/o infection auto recessive inheritance in persons of Mediterranean origin fever; serosal surfaces affected
Familial Mediterranean Fever
31
What is the link btw Alzheimers and Down Syndrome?
the beta-amyloid precursor protein is on chromosome 21 and DS = trisomy 21 early Alzheimers seen in DS individuals
33
What are the features of Familial Mediterranean Fever?
dysfunction of the neutrophils causing activation w/o infection auto recessive inheritance in persons of Mediterranean origin fever; serosal surfaces inflamed
34
What is the amyloid that is a byproduct of insulin that can deposit in the islets of the pancreas?
amylin
36
What is a misfolded protein that deposits into the extracellular space called?
an amyloid
37
How is medullary carcinoma of the thyroid definitively diagnosed?
fine needle aspiration of the thyroid
39
What is AL amyloid derived from?
Ig light chain
40
Where does amyloid most commonly deposit? What does this cause?
the kidney; nephrotic syndrome
41
What is restrictive cardiomyopathy?
stiffened heart can't dilate as well or pump properly, leading to HF
43
Systemic amyloidosis is divided into ____ and ____ types.
1a; 2a
44
What is the difference btw familial amyloid cardiomyopathy and senile cardiac amyloidosis ?
familial = mutated serum transthyretin senile = non-mutated serum transthyretin
46
2a amyloidosis can result from a \_\_\_\_\_, like autoimmune disease (SLE, RA, Crohn's, etc), \_\_\_\_\_\_, or \_\_\_\_\_.
chronic inflammatory state; malignancy; Familial Mediterranean fever
48
Why are amyloids bad?
they damage tissues
50
What can happen to the heart in amyloidosis?
restrictive cardiomyopathy arrhythmia
51
Where does amyloid tend to deposit?
around BVs
53
Where does amyloid most commonly deposit? What does this cause?
the kidney; nephrotic syndrome
54
What are non-mutated serum transthyretin deposits in the heart called?
senile cardiac amyloidosis
56
Amyloid deposition can be _____ or \_\_\_\_\_.
systemic; localized
57
What is the difference btw familial amyloid cardiomyopathy and senile cardiac amyloidosis ?
familial = mutated serum transthyretin senile = non-mutated serum transthyretin
58
How are the affected organs treated in amyloidosis?
no tx- they have to be transplanted
59
What kind of amyloidosis is dialysis-associated?
localized
60
2a amyloidosis can result from a \_\_\_\_\_, like autoimmune disease (SLE, RA, Crohn's, etc), or \_\_\_\_\_.
chronic inflammatory state; malignancy
61
What is AL amyloid derived from?
Ig light chain
62
What is the link btw Alzheimers and Down Syndrome?
the beta-amyloid precursor protein is on chromosome 21 and DS = trisomy 21 early onset Alzheimers seen in DS individuals
63
What is Beta-2-microglobulin?
a supportive protein for MHCI expression
64
What is serum transthyretin?
the 2nd most common protein in the blood
65
What is dialysis-associated amyloidosis?
beta-2-microglobulin deposits in joints
66
What is AA amyloid?
an amyloid derived from SAA
67
How is amyloidosis definitively diagnosed?
a tissue biopsy (usu a fat pad- abdominal or rectal)
68
What is a large loss of protein (\>3g in 24 hours) in the urine called?
nephrotic syndrome
69
What is medullary carcinoma of the thyroid?
a thyroid tumor derived from C cells, leading to overproduction of calcitonin, leading to amyloid deposition in the thyroid
70
Why does dialysis-assoc amyloidosis occur?
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
Where is the beta-amyloid precursor protein gene located?
on chromosome 21
72
What can happen to the heart in amyloidosis?
restrictive cardiomyopathy arrhythmia
73
What is localized amyloidosis?
amyloid deposition localized to a single organ or tissue
74
What are non-mutated serum transthyretin deposits in the heart called?
senile cardiac amyloidosis
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