AMYLOIDOSIS

Question 1

Techniques used for diagnosis in suspected cases of AL amyloidosis due to monoclonal plasma cell proliferations

Answer 1

1. Bone marrow biopsy
2. Serum immunoelectrophoresis (SIEP) and urine immunoelectrophoresis (UIEP)

Question 2

Type of amyloid deposited in the condition described below:

a. Armenian, Sephardic Jewish, and Arabic origins
b. attacks of fever
c. inflammation of serosal surfaces

Answer 2

AA

Question 3

1. Major amyloid fibril protein associated with chronic inflammatory conditions?
2. What is the chemically related precursor protein?

Answer 3

1. AA
2. SAA

Question 4

most common clinical presentaion of hemodialysis associated amyloidosis

Answer 4

carpal tunnel syndrome

Question 5

List 5 examples of conditions associated with deposition of AA amyloid?

Answer 5

1. Rheumatoid arthritis
2. Heroin abusers (sc injection)
3. Crohn disease
4. solid tumors- renal cell carcinoma and Hodgkin lymphoma.
5. Chronically infected burns, decubitus ulcers

Question 6

Appearance of amyloid on light microscopy on an H & E section

Answer 6

amorphous, eosinophilic, hyaline, extracellular

Question 7

1. Major amyloid fibril protein associated with isolated atrial amyloidosis
2. What is the chemically related precursor protein?

Answer 7

1. AANF
2. Atrial natriuretic factor

Question 8

1. Special stain for amyloid
2. Describe the appearance of amyloid when stained with Congo Red under an ordinary light microscope

Answer 8

1. Congo Red
2. pink or red color to amyloid deposits

Question 9

Most common location for deposition of amyloid in the form associated with plasma cell proliferation

Answer 9

perivascular and vascular deposits

Question 10

1. Major amyloid fibril protein associated with hemodialysis?
2. What is the chemically related precursor protein?

Answer 10

1. Aβ2M
2. β2-microglobulin

Question 11

1. Major amyloid fibril protein associated with Type 2 diabetes
2. What is the chemically related precursor protein?

Answer 11

1. AIAPP
2. Islet amyloid peptide

Question 12

Amyloid deposition is intracellular or extracellular?

Answer 12

Extracellular

Question 13

Primary underlying mechanism in pathogenesis of amyloidosis

Answer 13

Protein misfolding

Question 14

Amyloidosis of the spleen primarily involving the splenic follicles, producing tapioca-like granules

Answer 14

Sago spleen

Question 15

Amyloidosis of the spleen primarily involving the walls of the splenic sinuses and connective tissue framework

Answer 15

Lardaceous spleen

Question 16

First site of deposition of amyloid

Answer 16

adjacent to basement membranes.

Question 17

Nuclear imaging technique used to diagnose amyloidosis

Answer 17

Scintigraphy with radiolabeled serum amyloid P (SAP)

Question 18

See the attached image. List the expected clinical consequences.

Answer 18

Proteinuria, nephrotic syndrome→ pitting edema/effusions; ↓oncotic pressure (↓albumin)

Renal failure

Question 19

1. Major amyloid fibril protein associated with multiple myeloma and other monoclonal plasma proliferations
2. What is the chemically related precursor protein ?

Answer 19

1. AL
2. Immunoglobulin light chains, chiefly lambda type.

Question 20

1. Major amyloid fibril protein associated with familial amyloidotic polyneuropathies
2. What is the chemically related precursor protein?

Answer 20

1. ATTR
2. Transthyretin

Question 21

Identify the type of amyloid likely to be deposited in the condition described below:

Elderly; male

Diastolic heart failure

arrhythmias

Answer 21

wild-type “senile” TTR amyloid

Question 22

Describe the appearance of amyloid when stained with Congo Red and viewed under polarizing light

Answer 22

apple-green birefringence

Question 23

3 points to describe the physical nature of amyloid as seen on electron microscopy

Answer 23

Rigid, continuous, nonbranching fibrils

approximately 7.5 to 10 nm diameter

cross-β-pleated sheet conformation

Question 24

Common sites of biopsy in systemic amyloidosis

Answer 24

omental fat pad, rectum, and gingiva

Question 25

Why does AL amyloidosis of vessels cause hemorrhage?

Answer 25

AL amyloid binds and inactivates factor X

Question 26

1. Major amyloid fibril protein associated with medullary carcinoma thyroid
2. What is the chemically related precursor protein?

Answer 26

1. A Cal
2. Calcitonin

Question 27

See the attached image and description below. Identify the type of amyloid likley to be deposited.

90 year old male with dementia

Answer 27

Alzheimer disease - Type of amyloid fibril protein is Aβ

Question 28

Precursor proteinassociated with cerebral amyloid deposits in Alzheimer disease

Answer 28

Amyloid precursor protein (APP)