Case 27 – update 5

Our patient has a small IgG lambda paraprotein along with plasma cells in the marrow.  She has orthostatic hypotension, peripheral neuropathy and heart failure. Because of this the team were keen to investigate the possibility of amyloidosis. In order to diagnose amyloid the congo red stain is used which when amyloid fibrils are present demonstrates apple green birefringence under polarised light microscopy.

Investigations to look for end organ damage include:

  • Urinalysis – proteinuria/light chain on electrophoresis.
  • FBC – anaemia – often mild, thrombocytopenia.
  • U&Es – raised creatinine.
  • LFTs – elevated ALP
  • Clotting – there is abnormal clotting in 50% of cases – raised INR, factor X deficiency.
  • Inflammatory markers – raised ESR, normal CRP.
  • ECG – small complexes
  • Echocardiography – ventricular thickening with reduced ventricle size.
  • NTproBNP and cardiac troponins

Questions:

  • How is amyloid treated?
  • How patient has suspected cardiac involvement as well as autonomic symptoms. Would this change your management?

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