10- AMYLOIDOSIS

    Cards (7)

    • Amyloidosis
      A group of disorders characterized by extracellular deposits of amyloid fibrils (a protein in abnormal beta-sheet fibrils form, resistant to degradation)
    • Amyloidosis
      • Normal sized kidneys
      • Nodular sclerosis (Kimmelstiel–Wilson) like diabetes
    • Types of amyloidosis

      • AL (primary) amyloidosis
      • AA (secondary) amyloidosis
    • AL Amyloidosis
      Primary amyloidosis, due to abnormal clonal proliferation of plasma cells leading to deposition of immunoglobulin (Ig) light chains: λ (75% of cases) or ҡ (associated with multiple myeloma)
    • AA Amyloidosis
      Reactive/secondary amyloidosis, due to chronic inflammation from conditions like rheumatoid arthritis, tuberculosis, osteomyelitis, or familial Mediterranean fever
    • Clinical features of primary amyloidosis
      • Renal: proteinuria, nephrotic syndrome or progressive renal failure
      • Heart: CHF
      • Intestine: malabsorption, hepatomegaly
      • Nerves: carpal tunnel syndrome, peripheral neuropathy
      • Skin: easy bruising
    • Diagnosis of amyloidosis involves positive Congo Red staining of amyloid deposits within the glomerulus with apple-green birefringence under polarized light microscopy, and electron microscopy showing randomly organized amyloid fibrils
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