Amyloidosis: types

Amyloidosis: types

• the most common form of amyloidosis
• L for immunoglobulin Light chain fragment
  Light chain fragment
  You've never been tested on this concept
  You've not yet rated this concept.
• due to myeloma
  myeloma
  You've never been tested on this concept
  You've not yet rated this concept.
  , Waldenstrom's, MGUS
• features include: nephrotic syndrome
  nephrotic syndrome
  You've never been tested on this concept
  You've not yet rated this concept.
  , cardiac and neurological involvement, macroglossia, periorbital eccymoses

• A for precursor serum amyloid A protein, an acute phase reactant
• seen in chronic infection/inflammation
• e.g. TB, bronchiectasis, rheumatoid arthritis
• features: renal involvement most common feature

• associated with patients on haemodialysis
• mechanism:
  • in patients on long-term haemodialysis (usually >5–10 years), β2-microglobulin, a component of MHC class I molecules, accumulates in the blood because it is not efficiently cleared by standard dialysis membranes.
  • over time, β2-microglobulin forms amyloid fibrils that deposit in joints, bones, and peri-articular tissues.
• typical clinical features:
  • carpal tunnel syndrome (very common)
  • joint pain and stiffness (especially shoulders, hips, wrists)

Pathophysiology of amyloidosis