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CP Test 2 > B4-096 Amyloidosis > Flashcards

B4-096 Amyloidosis Flashcards

1
Q

amyloid plaques stain what color?

A

apple-green birefringence

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2
Q

what stain is used to visualize amyloid plaques?

A

congo red

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3
Q

amyloid plaques form [….] sheets

A

beta pleated

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4
Q

what molecule causes the native proteins to stick together?

A

heparin sulfate

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5
Q

AL is caused by

A

more cells making protein

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6
Q

ATTR is caused by

A

unstable protein in the native state

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7
Q

aggregation is […] and […]

A

slow; self catalyzing

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8
Q

caused by monoclonal light chains

A

AL

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9
Q

seen in plasma cell disorders

A

AL

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10
Q

seen in chronic inflammatory conditions

A

AA

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11
Q

familial mediterranean fever

A

AA

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12
Q

seen in patients with end stage renal disease/long term dialysis

A

beta 2 microglobulin

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13
Q

TTR is produced by

A

the liver and chorid plexus

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14
Q

carries T4 and retinol

A

TTR

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15
Q

TTR normally exists as a

A

tetramer

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16
Q

in ATTR, amyloid forms as a result of

A

dissociated TTR monomers

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17
Q

TTR

A

transthyretin

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18
Q

primarily affects peripheral nerves and heart

A

ATTRv

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19
Q

decreased protein stability, proteins more readily dissociate

A

ATTRv

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20
Q

caused by germline mutations

A

ATTRv

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21
Q

which affected tissue has the worse long term prognosis?

A

heart

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22
Q

caused by both increased concentration and loss of protein stability as a result of aging

A

ATTRwt

senile

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23
Q

primarily affects heart

A

ATTRwt

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24
Q

AL is primarily caused by aggregation of what type of light chains

A

lambda

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25
Q

increased concentration due to neoplastic plasma cell clone

A

AL

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26
Q

in systemic AL, the clone is usually in

A

bone marrow

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27
Q

in localized AL, the clone is typically in

A

tissue

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28
Q

primarily affects heart and kidney

A

AL

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29
Q

AL light chains are

A

highly toxic

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30
Q

Abeta2m is decreasing in prevalence due to

A

improved dialysis filters

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31
Q

mainly affects osteoarticular tissues

A

Abeta2m

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32
Q

serum amyloid A protein is an

A

acute phase reactant

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33
Q

primarily affects kidney

A

AA

34
Q

what has reduced the prevalence of AA in developed countries?

A

eradication of chronic infection and improved treatment of autoimmune diseases

35
Q

clinical severity depends on

A

tissue tropism

36
Q

examples of local, benign amyloidosis

A
  • insulin injection site
  • seminal vesicle
  • islet amyloid pancreatic
37
Q

primary sources of mortality of amyloidosis

A

cardiomyopathy
renal failure
coagulopathy

38
Q

coagulopathy: amyloidosis causes both […] and […] disorders

A

bleeding; clotting

39
Q

amyloid and precursor proteins bind […] causing bleeding

A

X

40
Q

amyloidosis coagulopathy can result in acquired

A

vWF deficiency

41
Q

clotting in amyloidosis is caused by

A

left atrial stasis and damage

risk of thrombi

42
Q

anticoagulation is only

A

partially protective

43
Q

how does amyloidosis affect the kidney?

A

infiltrates glomerulus –> proteinuria –>nephrotic syndrome –> renal failure

44
Q

bone and soft tissue red flags for amyloidosis

A
  • carpal tunnel
  • spinal stenosis
  • biceps tendon rupture
  • fracture
  • macroglossia (AL)
45
Q

red flags for amyloidosis in the nervous system

A
  • peripheral neuropathy (ATTRv esp.)
  • syncope
  • gastroparesis
46
Q

muscalaris mucosae infiltration leads to gastrophoresis in

A

AL

47
Q

mucosal infiltration leads to malabsorption in

A

AA

48
Q

as contractility in the heart fails, there is no ability for LV dilation to preserve EF causing

A

reliance on heart rate

49
Q

drugs poorly tolerated in amyloidosis

A
  • beta blockers
  • ACE/ARB
  • digoxin
  • Ca+ channel blockers
50
Q

drugs well tolerated in amyloidosis

A
  • loop diuretics
  • aldosterone antagonists
51
Q

cardiomyopathy is characterized by

A
  • HFpEF
  • restrictive physiology
  • arrhythmias
52
Q

massively underdiagnosed

A

ATTRv

25% penetrance

53
Q

average time from symptoms to diagnosis for amyloidosis is

A

2 years

54
Q

what condition has a 100% correlation with amyloid deposits

A

lumbar spinal canal stenosis

ligementum flavum

55
Q

classic ECG for cardiac amyloidosis

A
  • low voltage
  • poor p wave progression
56
Q

common echo findings for cardiac amyloidosis

A
  • biatrial enlargement
  • thickened spetum
  • concentric LVH
  • hyperechoic

basically, infiltrative disease

57
Q

low voltage ECG + increased LV septum…think…

A

infiltrative disease

58
Q

what finding on cardiac MRI is both sensitive and specific for cardiac amyloidosis?

A

late gadolinium enhancement

59
Q

on strain imaging, cardiac amyloidosis shows

A
  • basal predominance
  • apical sparing
  • unique acoustic speckles
60
Q

what major findings are highly specific for cardiac amyloidosis

A
  • apical sparing on strain echo
  • infiltrative pattern on MRI
61
Q

serum free light chain assay is very sensitive for

A

AL

62
Q

qualitative for disease monitoring of AL

A

serum free light chain assay

63
Q

grade 2-3 PYP scan is diagnostic of

A

ATTR

64
Q

next best step following

  • amyloid suspected on clinical findings
  • monoclonal protein present
  • PYP grade 1-3
A

cardiac biopsy

definitely AL, ATTR has not been excluded based on PYP grading

65
Q

next best step following

  • amyloid suspected on clinical findings
  • monoclonal protein present
  • PYP grade 0
A
  • AL
  • consider non-cardiac biopsy

ATTR is excluded based on PYP grading

66
Q

next best step following:

  • amyloid suspected on clinical findings
  • monoclonal protein excluded
  • PYP grade 2-3
A
  • ATTR diagnosed
  • TTR genotyping
67
Q

next best step following

  • amyloid suspected on clinical findings
  • monoclonal protein excluded
  • PYP grade 1
A

cardiac biopsy

68
Q

next best step following

  • amyloid suspected on clinical findings
  • monoclonal protein excluded
  • PYP grade 0
A

excludes AL and ATTR, consider alternate diagnosis

69
Q

mainstay treatment for ATTR

A

tafamidis

causes less dissociation

70
Q

treatment option for ATTRv

A

liver transplant

71
Q

management of ATTR is based on 3 options

A
  • TTR reduction
  • stabilization
  • fibril degradation
72
Q

TTR reduction is only available for

A

ATTRv

73
Q

ideal therapy for AL

A

stem cell transplant

many patients are ineligible due to cormorbidities

74
Q

associated with chronic inflammation from chronic infection or autoimmunity and frequently involves kidneys

A

AA

75
Q

stabilizes TTR tetramers

A

tafamidis

76
Q

prevents fibril formation and is protective against damage by TTR monomers

A

doxycycline

77
Q
  • cause mRNA degradation
  • interfere with translation of TTR
A

patisiran
inotersan

78
Q

silences the TTR gene and reduces serum TTR concentrations

A

patisiran/inotersan

79
Q

will eliminate TTRv but not affect overall levels of TTR

A

liver transplant

80
Q

stabilizes TTR but does not reduce levels

A

tafamadis

CP Test 2 (16 decks)

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