Table 4.

Supportive therapy in AL amyloidosis

ConditionSupportive measures
Fluid retention 
  • Salt restriction

  • Careful administration of diuretics (furosemide or torasemide, spironolactone)

  • Liberal use can worsen hypotension and reduce pre-load which can exacerbate cardiac dysfunction

 
Hypotension 
  • Fitted elastic stockings

  • Midodrine, droxidopa, and pyridostigmine

 
Neuropathy 
  • Gabapentin and pregabalin

  • Serotonin-norepinephrine reuptake inhibitors (duloxetine and venlafaxine)

 
Diarrhea 
  • Loperamide

  • Octreotide in patients who are refractory to loperamide

 
Malnutrition 
  • Nutritional counseling and support

 
End-stage renal disease 
  • Dialysis

  • Renal transplant in eligible patients with adequate hematologic response (CR/VGPR)101 

 
Heart failure 
  • Beta-blockers, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and calcium channel blockers should be used with great caution in cardiac amyloidosis

  • Heart transplant in eligible young patients with severe heart damage and with minimal or no extracardiac involvement. This should be followed by effective chemotherapy. Risk-adapted chemotherapy while on transplant list is recommended under intensive monitoring

 
ConditionSupportive measures
Fluid retention 
  • Salt restriction

  • Careful administration of diuretics (furosemide or torasemide, spironolactone)

  • Liberal use can worsen hypotension and reduce pre-load which can exacerbate cardiac dysfunction

 
Hypotension 
  • Fitted elastic stockings

  • Midodrine, droxidopa, and pyridostigmine

 
Neuropathy 
  • Gabapentin and pregabalin

  • Serotonin-norepinephrine reuptake inhibitors (duloxetine and venlafaxine)

 
Diarrhea 
  • Loperamide

  • Octreotide in patients who are refractory to loperamide

 
Malnutrition 
  • Nutritional counseling and support

 
End-stage renal disease 
  • Dialysis

  • Renal transplant in eligible patients with adequate hematologic response (CR/VGPR)101 

 
Heart failure 
  • Beta-blockers, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and calcium channel blockers should be used with great caution in cardiac amyloidosis

  • Heart transplant in eligible young patients with severe heart damage and with minimal or no extracardiac involvement. This should be followed by effective chemotherapy. Risk-adapted chemotherapy while on transplant list is recommended under intensive monitoring

 

See Cibeira et all.70 

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