(P2) Dis. of Imm: Amyloidosis (the kidney) (H/E) (362); Congo Red (374)

Question 1

1. Name this slide:
2. Name the Stain:
3. Define this condition:

Answer 1

1. Amyloidosis (the kidney)

2. H/E stain

3. It is a conditon associated with a number of inherited and inflammatory disorders in which exracellular deposits of fibrillar proteins are responsible for tissue damage and functional compromise.

Question 2

Amyloid

Define its character:

Answer 2

is a complex of abnormal fibrils which are produced by the aggregation of misfolded proteins (which are soluble in their normal folded configurtion)

• TO SIMPLIFY:*
• group of diverse extravascular/extracellular protein deposits that have;*
• 1. common pathomorphological properties*
• 2. affinities for specific dyes*
• 3. characteristic appearance under polarized light.*

Question 3

1. NAME THIS SLIDE
2. This disease is primary or Secondary?
3. It is associated with depositions of what material into where?

Answer 3

1. Amyloidosis (the kidney)

2. Secondary, non-inflammatory glomerulopathy

3. Associated with

Amyloid depositions within glomeruli

(sometimes other parts of the kidney)

Question 4

Amyloidosis (the kidney)

• What 2 types of Amyloidosis*
• can be found in the kidneys?*

Answer 4

1. AL- (Amyloid light chain) deposition of light chains of immunoglobulins
2. AA- (Amyloin-Associated) deposition of
   * Alpha-amyloid* -amyloid fibril protein is derived from a unique non-Ig protein made by the liver

Question 5

NAME THIS SLIDE

CLINICAL PRESENTATION:

Answer 5

Amyloidosis (the kidney)

1. PROTEINURIA
2. RENAL INSUFFICIENCY
3. NEPHROTIC SYNDROME

Question 6

NAME THIS SLIDE :)

Microscopic finding:

Answer 6

Amyloidosis (the kidney)

1. Amyloid depositions within glomeruli

(sometimes depsitions may be found in renal parenchyma and within wall of blood vessels)

2. In advanced disease lumina of arterioles in glomerulus and urinary space of Bowmans Capsule are narrowed

Question 7

NAME THIS SLIDE

Name all structures that apply

Answer 7

Amyloidosis (the kidney)

Question 8

• Amyloidosis (the kidney)*
• What is the chemical nature of the red material seen on this slide?*

Answer 8

fibril proteins

+

glycogen

Question 9

Most commonly affected organs:

Answer 9

kidneys

heart

liver

GI

Question 10

The type of protein involved?

Answer 10

ATTR (transthyretin- TTR)

• ATTR amyloidosis is a form of systemic amyloidosis* caused by amyloid deposits made up of a protein called transthyretin (TTR).
• ATTR amyloidosis can be either hereditary or acquired (non-hereditary).*

TTR is always present in the bloo_d, where it _transports thyroid hormone and vitamin A (retinol), hence the name: ‘trans-thy-retin’.

All the TTR in the blood is produced by the liver. TTR in the brain and the eye is made separately by a structure called the choroid plexus, which is located within the brain and produces the cerebrospinal fluid that bathes the brain and spinal cord.

Question 11

NAME THIS SLIDE

Location of proteins?

Answer 11

Amyloidosis (the kidney)

extracellular

Question 12

NAME THIS SLIDE

1. define structures:
2. What is happening to the Glomerular architecture?
3. What is happening to the nucleus
4. deposits of amyloid can be recognized as

Answer 12

• Amyloidosis (the kidney)*
• 2. Amyloid in glomerulus (homogenous mass) Glomerular enlargement, architecture and c_apillary obliteration_, hypocellularity*
     
     3. nucleus is being squeezed to the side
     4. deposits of amyloid (pink around area)

Question 13

NAME THIS SLIDE

and

STAIN

Answer 13

Amyloidosis (the kidney)

(Congo red)

Question 14

NAME THE STAINING

AMYLOID STAINS WHAT COLOR

Answer 14

CONGO RED

AMYLOID stains “RED”

Question 15

Name this slide and Stain

1. define the red-structure within the glomerulus
2. what diagnostic method should be applied to this slide to confirm the nature of this lesion?

Answer 15

Amyloidosis (the kidney) (Congo Red)

1. Amyloid
2. polarized light with the use of

Thioflavin T (flourescent dye) for amyloid-under polarized light

Review: Thioflavins are fluorescent dyes that are available as at least two compounds, namely Thioflavin T and Thioflavin S. Both are used for histology staining and biophysical studies of protein aggregation

Question 16

Q. What diagnisitc method shold be applied to this slide to confirm the nature fo this lesion?

Answer 16

Congo Red

Question 17

Q. What is a chemical nature of the red material seen on this slide?

Answer 17

A: Protein (Fibril protein + Glycoprotein)

Question 18

Name other Histological Stains that can be used for Amyloidosis

Answer 18

1. Thioflavin (fourescent dye)
2. Methyl violet
3. Alcian blue