Chapter 10 - Amyloidosis Flashcards

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1
Q

Amyloidosis is caused by…

A

Abnormal aggregation of proteins into β-pleated linear sheets

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2
Q

Systemic types of Amyloidosis

A

Primary
Secondary
Dialysis-related

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3
Q

Primary Amyloidosis: associated Fibril protein

A

AL fibrils, derived from immunoglobulin Light chains

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4
Q

Secondary Amyloidosis: associated Fibril protein

A

Serum Amyloid A (AA)

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5
Q

Dialysis-related Amyloidosis: associated Fibril protein

A

B2-microglobulin

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6
Q

Primary Amyloidosis is seen in…

A

Plasma cell disorders like Multiple Mieloma

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7
Q

Secondary Amyloidosis is seen in…

A

Chronic inflammatory conditions: rheumatoid arthritis, IBD, familial Mediterranean fever, protracted infection

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8
Q

Systemic manifestations of Amyloidosis:

A

Heart failure

Macroglossia, hepatomegaly

Nephrotic syndrome

Easy bruising, splenomegaly

Neuropathy

Carpal tunnel syndrome

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9
Q

Amyloidosis in Alzheimer’s disease: associated fibril protein

A

β-amyloid

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10
Q

β-amyloid protein: origin

A

Amyloid precursor protein (APP)

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11
Q

Amyloidosis in DM2: associated fibril protein

A

Islet amyloid polypeptide (IAPP)

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12
Q

Amyloidosis causes DM2 by…

A

Deposition of amylin in pancreatic islets

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13
Q

Amyloidosis in Medullary thyroid cancer: associated fibril protein

A

Calcitonin

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14
Q

Amyloidosis in Isolated atrial amyloidosis: associated fibril protein

A

Atrial natriuretic peptide

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15
Q

Age-related amyloidosis: associated fibril protein

A

Normal (wild-type) transthyretin

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16
Q

Normal (wild-type) transthyretin is seen predominantly in…

A

Cardiac ventricles

17
Q

Forms of Hereditary Amyloidosis:

A

Familial amyloid cardiomyopathy

Familial amyloid polyneuropathies

18
Q

Hereditary Amyloidosis: associated fibril protein

A

Mutated transthyretin

19
Q

Transthyretin gene mutation causes…

A

Familial amyloid polyneuropathies

20
Q

Familial amyloid cardiomyopathy is caused by…

A

Ventricular endomyocardium deposition

21
Q

What stains and microscopic techniques can be used to visualize amyloid deposits?

A

Congo red staining, polarized light showing apple-green birefringence, and H&E staining

22
Q

What findings can be noted on light microscopy in a patient with amyloidosis?

A

Enlarged tubular basement membranes

23
Q

Isolated atrial amyloidosis increases the risk of…

A

Atrial fibrilation