Immunoglobulin-Related Amyloidosis Guidelines

Updated: Nov 30, 2019
  • 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 [37] :

  • Treatment should be initiated 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, renal function, and physiologic age.
  • Dose-attenuated conditioning chemotherapy with ASCT is not recommended outside of a clinical trial
  • Induction therapy before ASCT for patients with 10% or greater bone marrow plasmacytosis
  • ASCT for patients undergoing hemodialysis is feasible, especially if renal allograft is being considered
  • For patients with underlying lymphoproliferative disease or IgM monoclonal protein, ASCT is a reasonable option
  • For patients ineligible for ASCT, standard chemotherapy regimen MDex is a valuable first-line option and thalidomide, cyclophosphamide, and dexamethasone are potential beneficial as first-line therapy
  • Cyclophosphamide, bortezomib, and dexamethasone (CyBorD) or bortezomib, melphalan, and dexamethasone can be used in newly diagnosed patients and in relapsed patients, but neither regimen has not been systematically studied