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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
Cardiac amyloidosis may present as
Conduction disturbances or restrictive cardiomyopathy
26
Cardiac amyloidosis typically begins
between myocardial fibers --> Pressure atrophy
27
Causes of Immunocyte Dyscriasis Amyloidosis
Multiple Myeloma --> Bence Jones Proteins
28
Chronic inflammatory disease most likely to cause Amyloidosis is
RA
29
Common organs other than Kidney, Liver Heart
Adrenals, Thyroid, Pituitary
30
Consitutive overproduction of IL-1 and persistent inflammation
GOF in pyrin -->
31
Deposition of AL type amyloid is associated with some types of
monoclonal B cell proliferation disorders
32
Deposition of b2-microglobulin amyloid in patients receiving long-term dialysis occurs most commonly in
Carpal Ligaments of Wrist
33
Dx Amyloidosis by
Biopsy
34
Dye used for Amyloidosis
Congo Red
35
Familial Amyloid Polyneuropathies
Mutations in TTR
36
Feature common to most cases of reactive systemic amyloidosis
Chronic Inflammation
37
First site of amyloid deposits
blood vessels
38
Frequently there are __ in periphery of localized amyloid masses
Lymphocytes and Plasma Cells
39
Gene for familial Mediterranean Fever
Pyrin (inflammasome protein)
40
GOF in pyrin -->
Consitutive overproduction of IL-1 and persistent inflammation
41
Gross changes of kidney in amyloidosis
Unchanged or abnormally large, pale, gray, and firm
42
Hepatic amyloidosis is most likely to correlate with elevation of serum
ALP
43
How to amyloid proteins fold
beta-pleated sheets
44
Immunocyte Dyscriasis w/ Amyloidosis is what type
AL, systemic
45
Lardaceous Spleen
Deposits principally involve sinuses --> Extend to splenic pulp --> Formation of large, sheetlike deposits
46
Liver amyloid deposits first appear in
Space of Disse
47
Major cause of symptoms in reactive systemic amyloidosis
Nephrotic syndrome: proteinuria
48
Mediterranean Fever
Attacks of fever accompanied by inflammation of serosal surfaces
49
Mediterranean Fever is associated with tissue involvement indistinguishable from
Reactive Systemic Amyloidosis
50
Most characteristic gross findings of cardiac amyloidosis
Gray-pink, dewdrop-like subendocardial elevations, particularly in atrial chambers
51
Most common classical causes of Reactive Systemic Amyloidosis
TB, Bronhiectasis, Chronic Osteomyelitis
52
Most common form of amyloidosis
Immunocyte Dyscriasis
53
Most common organ involved in amyloidosis
Kidney
54
Most common presenting manifestations
Nonspecific: weakness, fatigue, weight loss
55
Most common type of Amyloidosis
AL
56
Most important tool for Dx of amyloidosis
Biopsy and Congo red staining
57
Most serious organ feature of amyloidosis
Kidney
58
Multiple myeloma can cause __ Amyloidosis
Immunocyte Dyscriasis (AL, primary)
59
Mutations in TTR
Familial Amyloid Polyneuropathies
60
Normal serum protein that binds and transports thyroxine and retinol
TTR
61
On histological examination, amyloid deposition is always
extracelllular and begins between cells
62
Patients with RA are particularly prone to develop
amyloidosis
63
Peri-Orbital Ecchymoses -->
Basal Skull Fracture, Peri-Orbital Amyloidosis
64
Polyarteritis Nodosa is associated with what Ab's
None
65
Primary Amyloidosis pattern
Associated with a monoclonal plasma cell proliferation
66
Protein in AB2m
B2-microglobulin
67
Reactive Systemic Amyloidosis is secondary to
associated inflammatory conditions
68
Reactive Systemic Amyloidosis may also occur in tumors not
derived from immune cells
69
Relatively specific sign of Amyloidosis
Peri-orbital edema and yellow-brown skin discoloration
70
Rounded extracellular plaques of loose eosinophilic material
A-beta amyloid in brain tissue
71
SAA is only elevated in amyloidosis
False, other inflammatory states
72
SAA synthesized in liver under influence of
IL-6 and IL-1
73
Sago Spleen
Deposits limited to splenic follicles --> Tapioca-like granules grossly
74
Secondary Amyloidosis pattern
Complication of underlying chronic inflammatory or tissue destructive process
75
Segmental transmural necrotizing vasculitis of small/medium arteries w/ fibrinoid necrosis and lesions at diff stages
Polyarteritis Nodosa
76
Senile Systemic Amyloidosis
TTR deposited in hear of aged persons; Structurally normal, but high concentration
77
Senile Systemic Amyloidosis
TTR deposited in hear of aged persons; Structurally normal, but high concentration
78
Senile Systemic Amyloidosis dominantly involves
the heart
79
Small amounts of amyloid may not be present until organ is painted with
Iodine and Sulfuric Acid --> Mahogony brown
80
T/F: 2/3 of patients with myeloma amyloidosis have bence jones proteins in serum
False, almost all
81
T/F: Amyloid deposits usually elicit inflammation
FALSE
82
T/F: Amyloidosis develops in only 10% of patients with myeloma who have free light chains
TRUE
83
T/F: Amyloidosis is usually an inflammatory condition
FALSE
84
T/F: Majority of patients with AL amyloid have multiple myeloma or b cell neoplasm
FALSE
85
T/F: Most of the time SAA is elevated, it will lead to AA amyloid deposits
FALSE
86
T/F: Primary (AL) amyloidosis can be distinguished from secondary by organ distribution
FALSE
87
TTR
Normal serum protein that binds and transports thyroxine and retinol
88
TTR amyloidosis presents with
Sudden Death, Dyspnea
89
Type of Amyloid in Familial Amyloidotic polyneuropathies
ATTR (Transthyretin)
90
Type of protein in Reactive Systemic Amyloidosis
AA
91
What causes TTR amyloidosis
Mutations; or Normal accumulations with aging
92
When amyloid accumulates in larger amounts, the organ frequently is
enlarged and appears gray with a waxy, firm consistency
93
Where is AB amyloid found
Cerebral plaques, Alzheimer's
94
Where to biopsy for general amyloidosis
Rectum or Gingiva
95
Whey cardiac amyloidosis is accompanied by systemic involvement, usually of the__ type
AL
96
With advent of effective antimicrobials, reactive systemic amyloidosis is seen most frequently in setting of
Chronic Inflammation by autoimmune states

Pathology 2 (9 decks)

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