Gen Path Exam 3 - Autoimmune Disease - Amyloidosis

Question 1

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

Answer 1

Amyloidosis

Question 2

What is each variety of amyloidosis identified by?

Answer 2

Immunochemical nature of amyloid protein fibrils

Question 3

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

Answer 3

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

Question 4

Amyloidosis is an important cause of what 3 things?

Answer 4

Nephrotic syndrome
Renal insufficiency
Progressive renal failure

Question 5

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

Answer 5

Amyloid

Question 6

What does amyloid always have?

Answer 6

Beta-pleated sheet configuration

Question 7

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

Answer 7

folding; fibrils

Question 8

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

Answer 8

Serum Amyloid P (SAP)

Question 9

Where is Serum Amyloid P (SAP) deposited?

Answer 9

Tissues

Question 10

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

Answer 10

Insoluble beta-pleated sheets

Question 11

What does 95% of amyloid material consist of?

Answer 11

Fibril proteins

Question 12

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

Answer 12

P component
Glycoproteins

Question 13

What do amyloid deposits cause?

Answer 13

Tissue injury

Question 14

What do amyloid deposits impair? How?

Answer 14

Normal function by causing pressure on cells/tissue

Question 15

Do amyloid deposits in tissues evoke an inflammatory response?

Answer 15

NO!

Question 16

What are the 3 most common forms of amyloid proteins?

Answer 16

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

Question 17

Which form of amyloid protein?

Derived from plasma cells

Answer 17

Amyloid light chain (AL)

Question 18

Which form of amyloid protein?

Contains immunoglobulin light chains

Answer 18

Amyloid light chain (AL)

Question 19

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

Answer 19

Amyloid-associated (AA)

Question 20

Which form of amyloid protein?

Found in cerebral lesions of pts with Alzheimer’s

Answer 20

AB amyloid

Question 21

Which form of amyloid protein?

Derived from transmembrane glycoprotein called amyloid precursor protein (APP)

Answer 21

AB amyloid

Question 22

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

Answer 22

extracellular; basement membranes

Question 23

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

Answer 23

Amorphous + eosinophilic

Question 24

What is amyloid stained by?

Answer 24

Congo red dye

Question 25

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

Answer 25

apple green birefringence; polarized

Question 26

What other techniques can be used to view amyloid?

Answer 26

Immunochemistry Fluorescence Metachromatic

Question 27

Primary or secondary amyloidosis? Also called immunocytic dyscrasia amyloidosis

Answer 27

Primary

Question 28

Primary or secondary amyloidosis? Caused by deposition of amyloid fibrils derived from immunoglobulin light chains (amyloid light chain protein (AL))

Answer 28

Primary

Question 29

Primary or secondary amyloidosis? Associated with plasma cell disorders such as multiple myeloma and Waldenstrom macroglobulinemia

Answer 29

Primary

Question 30

Primary or secondary amyloidosis? Amyloid deposition in tissues of mesodermal origin (heart, muscle, tongue)

Answer 30

Primary

Question 31

Primary or secondary amyloidosis? May involve the kidney; amyloid deposits in glomerular mesangium and interstitial tissue btwn tubules

Answer 31

Primary

Question 32

Where does amyloid deposition occur in primary amyloidosis?

Answer 32

Tissues of mesodermal origin (heart, muscle, tongue)

Question 33

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

Answer 33

Secondary

Question 34

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

Answer 34

Chronic tissue destruction/inflammation

Question 35

Primary or secondary amyloidosis? Complication of chronic inflammatory disease (RA, TB, osteomyelitis, syphilis, leprosy)

Answer 35

Secondary

Question 36

Primary or secondary amyloidosis? May complicate non-inflammatory disorders (renal cell carcinoma, Hodgkin's disease)

Answer 36

Secondary

Question 37

Primary or secondary amyloidosis? Involves parenchymatous organs like the kidney, liver, adrenals, pancreas, lymph nodes, and spleen

Answer 37

Secondary

Question 38

Primary or secondary amyloidosis? Nephrotic syndrome is very common

Answer 38

Secondary