Table 3

Treatments for patients with cardiac amyloid light chain (AL) amyloidosis and clinical outcome

Treatment Mechanism of action Clinical outcome
Plasma cell-directed therapiesEliminate the production of amyloidogenic LCPatients with advanced cardiac amyloidosis often die despite HR, whereas patients with less severe cardiac involvement may experience rapid improvement in cardiac biomarkers and cardiac function when FLC can be reduced by 50%–90%7
Cardiac support therapies and/or heart transplantationAmeliorate symptoms of heart failure while patient is treated with other therapies or awaiting heart transplantationPatients with isolated cardiac deposition of amyloid may respond best to transplantation, but these treatments are supportive and not disease modifying
Amyloid-directed therapiesInhibit amyloid fibril formation or neutralise and clear circulating and deposited LC aggregatesPublished clinical trial data have demonstrated cardiac and other organ responses with anti-LC45 and anti-SAP47 immunotherapy
Preliminary data suggest a potential survival benefit of doxycycline
  • FLC, free light chain; HR, haematological response; LC, light chain; SAP, serum amyloid P component.