Amyloidosis Flashcards

1
Q

What is amyloidosis?

A

extracellular deposition of insoluble fibrillar protein termed “amyloid”

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2
Q

What does the accumulation of amyloid cause?

A

tissue/organ dysfunction

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3
Q

Name the ways in which amyloidosis can be classified

A

LOCATION
- systemic or localized

PRECURSOR PROTEIN
(e.g. AL in myeloma - A for Amyloid, L for Light chain)

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4
Q

Ix to diagnose amyloidosis

A
  • Congo red staining: apple-green birefringence
  • serum amyloid precursor (SAP) scan
  • biopsy of skin, rectal mucosa, or abdominal fat
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5
Q

Positive amyloidosis finding on congo red staining

A

apple-green birefringence

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6
Q

Name the subtype:

Most common form
L = Light chain fragment
(Myeloma, Waldenstrom’s, MGUS)

Features:
- nephrotic syndrome
- cardiac/neuro involvement
- macroglossia
- periorbital eccymoses

A

AL Amyloidosis

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7
Q

Name the subtype:
Precursor serum amyloid A protein (acute phase)

  • Seen in chronic infection/inflammation
    e.g. TB, bronchiectasis, RA

Features:
- renal involvement most common

A

AA Amyloidosis

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8
Q

Name the subtype

Precursor protein is beta-2 microglobulin, part of the major histocompatibility complex

Usually associated with patients on renal dialysis

A

Beta-2 microglobulin amyloidosis

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9
Q
A
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