Updated: 2/12/2018

Amyloidosis

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Overview
  • Amyloid is misfolded protein that takes the form of a beta-pleated sheet
    • the protein cannot be degraded by cellular enzymes
    • results in accumulation in the extracellular space
    • the deposited mass of the misfolded protein is damaging to tissues
  • Diagnosis made by demonstrating 
    • apple-green birefringence of Congo red stain under polarized light 
  • Amyloid can form from a variety of proteins but the above characterisitic are shared properties
  • Amyloid can be localized or systemic
Systemic Amyloidosis
  • Affects the entire body and can be of primary or secondary cause
  • Primary
    • result of AL amyloid deposition
      • derived from Ig light chain
      • classically seen in multiple myeloma
  • Secondary
    • result of AA amyloid deposition
      • derived from acute phase reaction of inflammation 
        • associated with increased levels of IL-6
      • can see with rheumatoid arthritis or other forms of chronic inflammation
  • Clinical findings
    • deposition in glomerulus
      • results in nephrotic syndrome
    • deposition in the heart
      • results in arrhythmias and/or restrictive cardiomyopathy
    • deposition in liver, spleen, tongue, intestine
Localized Amyloidosis
  • Affects only a specific organ
Types
Protein
Derived from
Senile cardiac
AF
Transthyretin
Isolated Atrial
AANF
Atrial Natriuretic Peptide (ANP 
Diabetes mellitus type 2
AE
Amylin
Medullary carcinoma of the thyroid
A-CAL
Calcitonin
Alzheimer's disease
β-amyloid
Amyloid precursor protein (APP)

Dialysis-associated

β2-microglobulin
MHC class I proteins
 

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