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Amyloidosis

extracellular deposition of pathologic insoluble fibrillar proteins in organs and tissues
amyloid protein typically beta-pleated sheets
multiple organs involved

familial
- Familial Transthyretin-associated amyloidosis (ATTR)
AL
- plasma cell dyscrasia
- related to Multiple Myeloma
- clonal dominance of amyloidogenic immunoglobulin light-chains
- causes
  - Multiple Myeloma
  - Waldenstom's macroglobulinaemia
  - Non-Hodgkins Lymphoma
Reactive systemic amyloidoses (AA)
- serum amyloid A (SAA) is acute phase protein
- related to chronic inflammatory disorders
- no macroglossia
- cardiac involvement rare
Local Amyloidosis
- in patients on long-term haemodialysis
- localised to various organs
- due to b₂-microglobulin

related to organs involved
kidneys
- proteinuria
- Nephrotic Syndrome
CNS
- Heart Failure
- cardiomyopathy
- bruising
CNS
- autonomic and sensory neuropathy common
- Carpal Tunnel Syndrome
- absence of CNS involvement
abdomen
- hepatomegaly
- splenomegaly
Macroglossia (20%)

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