[B] 1.31 Amyloidosis Flashcards

1
Q

Etymology: “Amyloid”

A

Amylum = Starch

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2
Q

Give the two main groups of amyloidosis pathogenesis

A
  • Immunmediated amyloidosis
  • Non-immunmediated amyloidosis
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3
Q

Morphology of an amyloid protein

A
  • Fibrils composed of protofilaments
  • Fibre network (β-sheets) unique in:
    • Staining
    • Optical characteristics
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4
Q

Amyloid physical properties

A
  • Formed by different proteins with abnormal structure
  • Structure change is the consequence of different effects
  • Insoluble in water & acids
  • Soluble in bases
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5
Q

Amyloidosis staining properties

A
  • HE: Pink, homogenous
  • Congo red: Orange
  • Polarised light: Golden shining
  • PAS: Red
  • Toluidine blue: Red
  • Immunohistochemistry: IgG
  • Macroscopic: Brown then blue
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6
Q

Other characteristics of amyloid

A
  • Structurlly stable amyloid
    • Trypsin resistant
    • (Unless structurally unstable)
  • 2 types of amyloidosis
    • Primary (Local & atypical)
    • Secondary (systemic & typical)
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7
Q

Immunmediated pathogenesis

A
  1. Background
  2. Monoclonal B-lymphocyte proliferation
  3. Plasma cell activation
  4. Immunoglobulin’s light chains
  5. Amyloid-light-protein

Result: Primary amyloidosis

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8
Q

Immunmediated pathogenesis

A
  1. Chronic inflammation
  2. Macrophage activation
  3. IL-1 & IL-6 production (cytokines)
  4. Abnormal protien production in hepatocytes
  5. SAA-protein (serum associated amyloid-protein) (soluble)
  6. AA-protein (insoluble)

Result: Secondary amyloidosis

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9
Q

Why is Secondary amyloidosis ‘secondary’

A
  • Preceded by:
    • Tissue necrosis
    • Chronic inflammation
    • Endotoxin production
  • In vaccinated animals with bacterium vaccine
  • In horses for hyperimmune serum production
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10
Q

Why is Secondary amyloidosis ‘typical’

A

Occurs on characteristic sites

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11
Q

Why is Secondary amyloidosis ‘systemic’

A

Observed in several organs

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12
Q

Recognition of amyloidosis

A
  • Mostly by macroscopic investigation
    • Local (predilection sites)
    • Systemic (Certain organs - liver, spleen, kideny)
  • Sometimes only by histopathology
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13
Q

Harmful effects of amyloid

A
  • Intercellular deposition
  • Blocks the normal transfer of materials
  • Loss of function, regressive changes in tissues

The latter changes cause the problems, not the amyloid itself

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14
Q

Pathology of systemic amyloidosis: Liver

A
  • Shape: Normal
  • Size: Enlarged
  • Colour: Pale, sometimes green in birds (biliverdin)
  • Consistency: Dry cut surface, easy to tear

Amyloid is deposited on the basal membrane of sinusoids or in the walls of blood vessels

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15
Q

Pathology of systemic amyloidosis: Spleen

A

Deposition in the vessels of the vascular elements of the Malpighi-bodies

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16
Q

Pathology of systemic amyloidosis: Kidneys

A

Deposition:

  • On the basal membrane of the glomerular capillaries and tubuli
  • On/in the wall of blood vessels of the interstitium
17
Q

Pathology of systemic amyloidosis: Adrenal gland

A

In the wall of the capillaries of the cortex

18
Q

Pathology of systemic amyloidosis: Intestines

A
  • On the basal membrane of the capillaries of the microvilli
  • In the intestinal glands
19
Q

Atypical, local amyloidosis

A
  • Atypical: Because the cause is unknown
  • Local: Because:
    • Occurs on the predilection sites of the body (“amyloid tumours”)
    • In the skin or in the mucosa of the ansal cavity of horses, sometimes tumour-like swellings
    • In the heart muscle of leptomeninx of aging animals
20
Q

APUD-amyloidosis

A

Amine precursor uptake and decarboxylation-amyloidosis

  • Production & decarboxylation of amine precursors
  • Result: Secreted into the cell’s environment → Amyloid produced from excess protein
21
Q

Cells of APUD-system

A
  • Enterochromaffin cells of stomach & intestinal wall
  • Parafollicular C-cells of thyroid gland
  • α- and β-cells of Langerhans-islets
  • ACTH-producing cells of adenohypophysis