Gen Path Exam 3 - Autoimmune Disease - Amyloidosis Flashcards

1
Q

Diverse family of rare, chronic infiltrative
localized or multisystem disorders characterized by the presence of extracellular deposits of insoluble fibrillar proteins

A

Amyloidosis

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2
Q

What is each variety of amyloidosis identified by?

A

Immunochemical nature of amyloid protein fibrils

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3
Q

What are the syndromes present at presentation from most to least common?

A

Nephrotic syndrome/renal failure
Carpal tunnel
CHF
Peripheral neuropathy
Orthostatic hypotension

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4
Q

Amyloidosis is an important cause of what 3 things?

A

Nephrotic syndrome
Renal insufficiency
Progressive renal failure

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5
Q

Not a single substance, but a group of substances that share a common physical structure that can be formed by a number of different proteins

A

Amyloid

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6
Q

What does amyloid always have?

A

Beta-pleated sheet configuration

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7
Q

Amyloidosis results from abnormal _________ of proteins, which are deposited as _________ in extracellular tissues and disrupt normal function

A

folding; fibrils

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8
Q

What is the soluble circulating protein found in the serum of pts with amyloidosis?

A

Serum Amyloid P (SAP)

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9
Q

Where is Serum Amyloid P (SAP) deposited?

A

Tissues

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10
Q

What is Serum Amyloid P (SAP) deposited in the tissues as?

A

Insoluble beta-pleated sheets

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11
Q

What does 95% of amyloid material consist of?

A

Fibril proteins

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12
Q

What does the other 5% of amyloid material consist of?

A

P component
Glycoproteins

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13
Q

What do amyloid deposits cause?

A

Tissue injury

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14
Q

What do amyloid deposits impair? How?

A

Normal function by causing pressure on cells/tissue

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15
Q

Do amyloid deposits in tissues evoke an inflammatory response?

A

NO!

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16
Q

What are the 3 most common forms of amyloid proteins?

A

Amyloid light chain (AL)
Amyloid-associated (AA)
AB amyloid

17
Q

Which form of amyloid protein?

Derived from plasma cells

A

Amyloid light chain (AL)

18
Q

Which form of amyloid protein?

Contains immunoglobulin light chains

A

Amyloid light chain (AL)

19
Q

Which form of amyloid protein?

Unique non-immunoglobulin protein derived from serum amyloid-associated protein (SAA) made by liver cells under influence of cytokines produced during inflammation

A

Amyloid-associated (AA)

20
Q

Which form of amyloid protein?

Found in cerebral lesions of pts with Alzheimer’s

A

AB amyloid

21
Q

Which form of amyloid protein?

Derived from transmembrane glycoprotein called amyloid precursor protein (APP)

A

AB amyloid

22
Q

Amyloid is ______________ in distribution, most
often appearing as accumulations proximate to ___________________

A

extracellular; basement membranes

23
Q

What is the appearance of amyloid in routine hematoxylin and eosin secretions?

A

Amorphous + eosinophilic

24
Q

What is amyloid stained by?

A

Congo red dye

25
Q

When stained with Congo red dye, amyloid demonstrates ________ _________ ______________ when viewed under ___________ light

A

apple green birefringence; polarized

26
Q

What other techniques can be used to view amyloid?

A

Immunochemistry
Fluorescence
Metachromatic

27
Q

Primary or secondary amyloidosis?

Also called immunocytic dyscrasia amyloidosis

A

Primary

28
Q

Primary or secondary amyloidosis?

Caused by deposition of amyloid fibrils derived from immunoglobulin light chains (amyloid light chain protein (AL))

A

Primary

29
Q

Primary or secondary amyloidosis?

Associated with plasma cell disorders such as multiple myeloma and Waldenstrom macroglobulinemia

A

Primary

30
Q

Primary or secondary amyloidosis?

Amyloid deposition in tissues of mesodermal origin (heart, muscle, tongue)

A

Primary

31
Q

Primary or secondary amyloidosis?

May involve the kidney; amyloid deposits in glomerular mesangium and interstitial tissue btwn tubules

A

Primary

32
Q

Where does amyloid deposition occur in primary amyloidosis?

A

Tissues of mesodermal origin (heart, muscle, tongue)

33
Q

Primary or secondary amyloidosis?

Deposition of fibrils w/ amyloid-associated protein (AA), which is formed by its precursor, serum amyloid-associated protein (SAA)

A

Secondary

34
Q

What leads to increased serum-amyloid associated protein (SAA)?

A

Chronic tissue destruction/inflammation

35
Q

Primary or secondary amyloidosis?

Complication of chronic inflammatory disease (RA, TB, osteomyelitis, syphilis, leprosy)

A

Secondary

36
Q

Primary or secondary amyloidosis?

May complicate non-inflammatory disorders (renal cell carcinoma, Hodgkin’s disease)

A

Secondary

37
Q

Primary or secondary amyloidosis?

Involves parenchymatous organs like the kidney, liver, adrenals, pancreas, lymph nodes, and spleen

A

Secondary

38
Q

Primary or secondary amyloidosis?

Nephrotic syndrome is very common

A

Secondary