Immunoglobulin-Related Amyloidosis Guidelines

Updated: Jun 28, 2022
  • Author: Slavomir Urbancek, MD, PhD; Chief Editor: Emmanuel C Besa, MD  more...
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Guidelines Summary

In 2015, Mayo Stratification of Myeloma and Risk-Adapted Therapy (mSMART) released consensus guidelines for the treatment of immunoglobulin light chain amyloidosis. Key recommendations include the following [39] :

  • Initiate treatment immediately in virtually all patients with systemic AL amyloidosis.
  • Consider high-dose chemotherapy with autologous stem cell transplant (ASCT) in selected patients based on troponin level, blood pressure, kidney function, and physiologic age.
  • Dose-attenuated conditioning chemotherapy with ASCT is not recommended outside of a clinical trial.
  • Induction therapy before ASCT is recommended for patients with 10% or greater bone marrow plasmacytosis.
  • ASCT for patients undergoing hemodialysis is feasible, especially if kidney transplantation is being considered.
  • For patients with underlying lymphoproliferative disease or IgM monoclonal protein, ASCT is a reasonable option.
  • For patients ineligible for ASCT, standard-dose melphalan and dexamethasone is a valuable first-line option and the combination of thalidomide, cyclophosphamide, and dexamethasone is potentially beneficial as first-line therapy.
  • Cyclophosphamide, bortezomib, and dexamethasone or bortezomib, melphalan, and dexamethasone can be used in patients with newly diagnosed or relapsed disease, but neither regimen has been systematically studied.