Sunday 21 November 2010

MRCP revision battle 56.1: Amyloidosis

7 non-waffling battles


MRCP revision battle 56.1: Amyloidosis
MRCP revision battle 56.2: Plasma exchange
MRCP revision battle 56.3: P450 inducers and inhibitors
MRCP revision battle 56.4: Therapeutic drug monitoring
MRCP revision battle 56.5: Vaccines
MRCP revision battle 56.6: Acute lymphoid leukaemia
MRCP revision battle 56.7: Nematodes = roundworms



MRCP revision battle 56.1: Amyloidosis

Amyloidosis is a pathological process characterised by extracellular accumulation of fibrils of insoluble protein.


Inherited forms of amyloidosis are rare but amyloid deposition is central to many diseases including Alzheimers and type 2 diabetes.


Types of amyloid:

1: AL = light chains
  • secreted by plasma B cells
  • occurs in 15% of myeloma patients
  • also occurs in:
    • Waldenstroms
    • MGUS
  • features:
    • cardiac deposition = 'sparkling' appearence on echo
    • GI 
    • macroglossia
    • peri orbital
  • median survival is 12 months
  • treatment is to treat the underlying condition, ?prednisolone

2: AA = serum amylase A
  • this is secondary amyloidosis, with the amyloid derived from serum amylase A which is an acute phase protein
  • seen in:
    • RA
    • TB
    • bronchiectasis
    • familial med fever
    • IBD
  • commonest organ involved = kidney  (congo red stain -->red-green birefrigence under polarised light)
  • median survival is 24 months

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