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Pathology 2 > Amyloidosis > Flashcards

Amyloidosis Flashcards

1
Q

Amyloidosis is fundamentally a disorder of

A

protein misofolding

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2
Q

3 most common amyloid proteins

A

AL, AA, AB

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3
Q

40% of patients with AL amyloid died of

A

Cardiac disease

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4
Q

A beta amyloidosis presents with

A

Dementia

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5
Q

AA amyloid is derived from

A

SAA, synthesized in liver

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6
Q

AA, AL, and ATTR can be distinguished from each other by

A

specific IHC staining

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7
Q

AB protein is derived from

A

APP

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8
Q

Abnormal pale color of heart muscle is characteristic of

A

Severe cardiac amyloidosis

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9
Q

AL amyloidosis is usually due to

A

Free Ig light chains due to a proliferation of plasma cells

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10
Q

AL amyloidosis most commonly presentes w/

A

Peripheral edema due to renal disease

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11
Q

AL protein is made up of

A

complete Ig light chains, amino terminal fragments of light chains, or both

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12
Q

AL protein is produced by

A

plasma cells

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13
Q

Amyloid deposition is seen in what percet of RA

A

3%

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14
Q

Amyloid deposits are also found where in kidney

A

Interstitial peritubular tissue, walls of blood vessels

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15
Q

Amyloid deposits are found principally where in kideny

A

Glomeruli

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16
Q

Amyloid in Carpal ligamentsof wrist

A

B2-microglobulin, patients on long-term dialysis

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17
Q

Amyloid in Mediterranean Fever

A

AA

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18
Q

Amyloid type in Senile Systemic Amyloidosis

A

TTR

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19
Q

Amyloidosis A =

A

Reactive Systemic Amyloidosis

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20
Q

Amyloidosis A is highly unlikely to present w/out

A

Hx of the preceding chronic inflammation disorder that caused it

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21
Q

Amyloidosis of “other organs” typically begins

A

in relation to stromal and endothelial cells

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22
Q

Amyloidosis that complicates course of patients on long-term dialysis

A

B2-microglobulin

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23
Q

B2-microglobulin is present in serum in high concentrations in patients with

A

renal disease

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24
Q

Cardiac amyloidosis is most often due to

A

TTR deposition

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25
Q

Cardiac amyloidosis may present as

A

Conduction disturbances or restrictive cardiomyopathy

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26
Q

Cardiac amyloidosis typically begins

A

between myocardial fibers –> Pressure atrophy

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27
Q

Causes of Immunocyte Dyscriasis Amyloidosis

A

Multiple Myeloma –> Bence Jones Proteins

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28
Q

Chronic inflammatory disease most likely to cause Amyloidosis is

A

RA

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29
Q

Common organs other than Kidney, Liver Heart

A

Adrenals, Thyroid, Pituitary

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30
Q

Consitutive overproduction of IL-1 and persistent inflammation

A

GOF in pyrin –>

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31
Q

Deposition of AL type amyloid is associated with some types of

A

monoclonal B cell proliferation disorders

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32
Q

Deposition of b2-microglobulin amyloid in patients receiving long-term dialysis occurs most commonly in

A

Carpal Ligaments of Wrist

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33
Q

Dx Amyloidosis by

A

Biopsy

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34
Q

Dye used for Amyloidosis

A

Congo Red

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35
Q

Familial Amyloid Polyneuropathies

A

Mutations in TTR

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36
Q

Feature common to most cases of reactive systemic amyloidosis

A

Chronic Inflammation

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37
Q

First site of amyloid deposits

A

blood vessels

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38
Q

Frequently there are __ in periphery of localized amyloid masses

A

Lymphocytes and Plasma Cells

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39
Q

Gene for familial Mediterranean Fever

A

Pyrin (inflammasome protein)

40
Q

GOF in pyrin –>

A

Consitutive overproduction of IL-1 and persistent inflammation

41
Q

Gross changes of kidney in amyloidosis

A

Unchanged or abnormally large, pale, gray, and firm

42
Q

Hepatic amyloidosis is most likely to correlate with elevation of serum

A

ALP

43
Q

How to amyloid proteins fold

A

beta-pleated sheets

44
Q

Immunocyte Dyscriasis w/ Amyloidosis is what type

A

AL, systemic

45
Q

Lardaceous Spleen

A

Deposits principally involve sinuses –> Extend to splenic pulp –> Formation of large, sheetlike deposits

46
Q

Liver amyloid deposits first appear in

A

Space of Disse

47
Q

Major cause of symptoms in reactive systemic amyloidosis

A

Nephrotic syndrome: proteinuria

48
Q

Mediterranean Fever

A

Attacks of fever accompanied by inflammation of serosal surfaces

49
Q

Mediterranean Fever is associated with tissue involvement indistinguishable from

A

Reactive Systemic Amyloidosis

50
Q

Most characteristic gross findings of cardiac amyloidosis

A

Gray-pink, dewdrop-like subendocardial elevations, particularly in atrial chambers

51
Q

Most common classical causes of Reactive Systemic Amyloidosis

A

TB, Bronhiectasis, Chronic Osteomyelitis

52
Q

Most common form of amyloidosis

A

Immunocyte Dyscriasis

53
Q

Most common organ involved in amyloidosis

A

Kidney

54
Q

Most common presenting manifestations

A

Nonspecific: weakness, fatigue, weight loss

55
Q

Most common type of Amyloidosis

A

AL

56
Q

Most important tool for Dx of amyloidosis

A

Biopsy and Congo red staining

57
Q

Most serious organ feature of amyloidosis

A

Kidney

58
Q

Multiple myeloma can cause __ Amyloidosis

A

Immunocyte Dyscriasis (AL, primary)

59
Q

Mutations in TTR

A

Familial Amyloid Polyneuropathies

60
Q

Normal serum protein that binds and transports thyroxine and retinol

A

TTR

61
Q

On histological examination, amyloid deposition is always

A

extracelllular and begins between cells

62
Q

Patients with RA are particularly prone to develop

A

amyloidosis

63
Q

Peri-Orbital Ecchymoses –>

A

Basal Skull Fracture, Peri-Orbital Amyloidosis

64
Q

Polyarteritis Nodosa is associated with what Ab’s

A

None

65
Q

Primary Amyloidosis pattern

A

Associated with a monoclonal plasma cell proliferation

66
Q

Protein in AB2m

A

B2-microglobulin

67
Q

Reactive Systemic Amyloidosis is secondary to

A

associated inflammatory conditions

68
Q

Reactive Systemic Amyloidosis may also occur in tumors not

A

derived from immune cells

69
Q

Relatively specific sign of Amyloidosis

A

Peri-orbital edema and yellow-brown skin discoloration

70
Q

Rounded extracellular plaques of loose eosinophilic material

A

A-beta amyloid in brain tissue

71
Q

SAA is only elevated in amyloidosis

A

False, other inflammatory states

72
Q

SAA synthesized in liver under influence of

A

IL-6 and IL-1

73
Q

Sago Spleen

A

Deposits limited to splenic follicles –> Tapioca-like granules grossly

74
Q

Secondary Amyloidosis pattern

A

Complication of underlying chronic inflammatory or tissue destructive process

75
Q

Segmental transmural necrotizing vasculitis of small/medium arteries w/ fibrinoid necrosis and lesions at diff stages

A

Polyarteritis Nodosa

76
Q

Senile Systemic Amyloidosis

A

TTR deposited in hear of aged persons; Structurally normal, but high concentration

77
Q

Senile Systemic Amyloidosis

A

TTR deposited in hear of aged persons; Structurally normal, but high concentration

78
Q

Senile Systemic Amyloidosis dominantly involves

A

the heart

79
Q

Small amounts of amyloid may not be present until organ is painted with

A

Iodine and Sulfuric Acid –> Mahogony brown

80
Q

T/F: 2/3 of patients with myeloma amyloidosis have bence jones proteins in serum

A

False, almost all

81
Q

T/F: Amyloid deposits usually elicit inflammation

A

FALSE

82
Q

T/F: Amyloidosis develops in only 10% of patients with myeloma who have free light chains

A

TRUE

83
Q

T/F: Amyloidosis is usually an inflammatory condition

A

FALSE

84
Q

T/F: Majority of patients with AL amyloid have multiple myeloma or b cell neoplasm

A

FALSE

85
Q

T/F: Most of the time SAA is elevated, it will lead to AA amyloid deposits

A

FALSE

86
Q

T/F: Primary (AL) amyloidosis can be distinguished from secondary by organ distribution

A

FALSE

87
Q

TTR

A

Normal serum protein that binds and transports thyroxine and retinol

88
Q

TTR amyloidosis presents with

A

Sudden Death, Dyspnea

89
Q

Type of Amyloid in Familial Amyloidotic polyneuropathies

A

ATTR (Transthyretin)

90
Q

Type of protein in Reactive Systemic Amyloidosis

A

AA

91
Q

What causes TTR amyloidosis

A

Mutations; or Normal accumulations with aging

92
Q

When amyloid accumulates in larger amounts, the organ frequently is

A

enlarged and appears gray with a waxy, firm consistency

93
Q

Where is AB amyloid found

A

Cerebral plaques, Alzheimer’s

94
Q

Where to biopsy for general amyloidosis

A

Rectum or Gingiva

95
Q

Whey cardiac amyloidosis is accompanied by systemic involvement, usually of the__ type

A

AL

96
Q

With advent of effective antimicrobials, reactive systemic amyloidosis is seen most frequently in setting of

A

Chronic Inflammation by autoimmune states

Pathology 2 (9 decks)

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