AP8: A

Question 1

Which of the following best defines amyloidosis? A. Intracellular accumulation of misfolded carbohydrates B. Extracellular deposition of insoluble fibrillar proteinaceous substance C. Intracellular deposition of starch-like granules in macrophages D. Extracellular accumulation of normal proteins in a liquid state

Answer 1

B. Extracellular deposition of insoluble fibrillar proteinaceous substance.

Question 2

The name “amyloid” was originally chosen because these deposits have staining characteristics similar to ______ although they are chemically unrelated.

Answer 2

Starch (amylose)

Question 3

Describe the fundamental pathogenesis of amyloidosis regarding cellular control mechanisms.

Answer 3

Amyloidosis occurs when normal control mechanisms fail. Misfolded proteins which are normally degraded intracellularly by proteasomes or extracellularly by macrophages instead aggregate to form insoluble fibrils that accumulate outside cells leading to tissue damage.

Question 4

Which of the following are the two broad categories of proteins that form amyloid? A. Normal proteins produced in excessive amounts with an inherent tendency to misfold B. Soluble proteins that become branched through enzymatic action C. Mutant proteins that are inherently prone to misfolding D. Native folded proteins that remain in their original tertiary structure

Answer 4

A C

Question 5

Approximately what percentage of amyloid material consists of fibril proteins? A. 5% B. 50% C. 75% D. 95%

Answer 5

D. 95%

Question 6

The most common form of amyloid associated with plasma cell disorders and consisting of immunoglobulin fragments is the ______ type.

Answer 6

AL (Amyloid light chain)

Question 7

In AL amyloidosis which light chain is found significantly more often than the other? A. Kappa B. Lambda C. Gamma D. Delta

Answer 7

B. Lambda

Question 8

Which of the following are characteristic of AA protein? A. It is a non-immunoglobulin protein B. It is synthesized primarily by the liver C. Its precursor is Serum Amyloid A D. It is found exclusively in Alzheimer’s disease

Answer 8

A B C

Question 9

Which protein is the precursor for the amyloid found in the cerebral plaques of Alzheimer’s disease? A. Transthyretin B. Serum Amyloid A C. Amyloid precursor protein D. β2-microglobulin

Answer 9

C. Amyloid precursor protein

Question 10

Amyloid found in patients on long-term hemodialysis consists of ______ which cannot be filtered through dialysis membranes.

Answer 10

β2-microglobulin (Aβ2m)

Question 11

What is the physical nature of amyloid as seen under electron microscopy?

Answer 11

It appears as continuous non-branching fibrils with a diameter of 7.5 to 10 nm with a cross β-pleated sheet conformation.

Question 12

Which rare chemical form of amyloid contributes to deposition by stabilizing fibrils and decreasing their clearance? A. Transthyretin B. Serum amyloid P component C. Misfolded prion proteins D. Immunoglobulin light chains

Answer 12

B. Serum amyloid P component

Question 13

Primary amyloidosis is a plasma cell disorder where the amyloid protein is of the ______ type.

Answer 13

AL

Question 14

What percentage of patients with multiple myeloma develop associated amyloidosis? A. 1% B. 5-15% C. 50% D. 100%

Answer 14

B. 5-15%

Question 15

Which inflammatory conditions are commonly associated with reactive systemic amyloidosis? A. Rheumatoid arthritis B. Ankylosing spondylitis C. Inflammatory bowel disease D. Acute viral influenza

Answer 15

A B C

Question 16

In reactive systemic amyloidosis inflammation leads to the release of interleukins ______ and ______ which stimulate the liver to synthesize SAA.

Answer 16

IL-6 and IL-1

Question 17

Describe the pathogenesis of AA amyloidosis in the context of chronic inflammation.

Answer 17

Chronic inflammation increases IL-6 and IL-1 leading to excessive SAA synthesis by the liver and failure of degradation resulting in AA deposition.

Question 18

Familial Mediterranean Fever follows which inheritance pattern? A. Autosomal dominant B. Autosomal recessive C. X-linked recessive D. Mitochondrial

Answer 18

B. Autosomal recessive

Question 19

Localized amyloidosis typically involves a single organ and may be surrounded by an infiltrate of ______ and plasma cells.

Answer 19

Lymphocytes

Question 20

In which endocrine tumors can microscopic deposits of localized amyloid be found? A. Medullary carcinoma of thyroid B. Islet tumors of pancreas C. Pheochromocytomas D. Pituitary adenomas

Answer 20

A B C

Question 21

Senile systemic amyloidosis primarily involves which organ? A. Liver B. Kidney C. Heart D. Spleen

Answer 21

C. Heart

Question 22

The amyloid found in senile systemic amyloidosis is composed of the normal ______ molecule.

Answer 22

TTR

Question 23

Which organs are predominantly involved in primary amyloidosis? A. Heart B. Gastrointestinal tract C. Kidneys D. Tongue

Answer 23

A B D

Question 24

Which organ is the most common and serious site of amyloid involvement in secondary amyloidosis? A. Spleen B. Liver C. Kidney D. Adrenals

Answer 24

C. Kidney

Question 25

Describe the macroscopic appearance of an organ heavily involved with amyloid.

Answer 25

Enlarged grey waxy firm lard-like organ

Question 26

On light microscopy amyloid appears as an amorphous hyaline extracellular deposit often found adjacent to the ______ membrane.

Answer 26

Basement

Question 27

In the kidney why might the organ eventually become shrunken? A. Excess protein synthesis B. Ischemia from vascular narrowing C. Loss of P component D. Reabsorption by macrophages

Answer 27

B. Ischemia from vascular narrowing

Question 28

Where in the kidney are amyloid deposits typically found? A. Glomeruli B. Interstitium C. Arterial walls D. Renal pelvis

Answer 28

A B C

Question 29

Which pattern of splenic amyloidosis produces tapioca-like granules? A. Lardaceous spleen B. Sago spleen C. Nutmeg spleen D. Cricket-ball spleen

Answer 29

B. Sago spleen

Question 30

In a lardaceous spleen amyloid involves the walls of the splenic ______ and the red pulp framework.

Answer 30

Sinuses

Question 31

Which nonspecific symptoms are often the first clinical manifestations of amyloidosis? A. Weakness B. Weight loss C. Lightheadedness D. Fever and chills

Answer 31

A B C

Question 32

What are the most common biopsy sites when systemic amyloidosis is suspected?

Answer 32

Kidney rectal tissue gingiva abdominal fat

Question 33

What is the characteristic Congo red staining appearance under polarized light? A. Pink B. Yellow fluorescence C. Apple-green birefringence D. Dark blue

Answer 33

C. Apple-green birefringence

Question 34

A rapid diagnostic test uses scintigraphy with radiolabelled ______.

Answer 34

Serum Amyloid P

Question 35

Which diagnostic method is confirmatory for amyloid fibrils? A. H&E B. Scintigraphy C. Polarized microscopy D. Electron microscopy

Answer 35

D. Electron microscopy

Question 36

Contrast prognosis of immunocyte-derived amyloidosis with and without myeloma.

Answer 36

Poor prognosis worse with myeloma

Question 37

In the liver amyloid deposits first appear in the ______.

Answer 37

Space of Disse

Question 38

Which organs are involved in Familial Mediterranean Fever? A. Kidney B. Spleen C. Blood vessels D. Brain

Answer 38

A B C

Question 39

Amyloidogenic potential depends on the ______ of the light chain.

Answer 39

Specific amino acid sequence

Question 40

In type II diabetes mellitus amyloid is found in the ______ of Langerhans.

Answer 40

Islets