Combined use of bortezomib, cyclophosphamide, and dexamethasone induces favorable hematological and organ responses in Japanese patients with amyloid light-chain amyloidosis: a single-institution retrospective study

Int J Hematol. 2015 Feb;101(2):133-9. doi: 10.1007/s12185-014-1705-9. Epub 2014 Nov 28.


Amyloid light-chain amyloidosis (ALA) is a rare disease with poor prognosis and is often associated with monoclonal gammopathy of undetermined significance, multiple myeloma, or Waldenström macroglobulinemia. Only high-dose melphalan with auto-peripheral blood stem cell transplantation (PBSCT) has shown high long-term hematological response rates, but combinations with novel agents, including bortezomib or lenalidomide, have recently shown high hematological response rates for AL amyloidosis patients. In the present study, we treated eight Japanese patients with AL amyloidosis using bortezomib, cyclophosphamide, and dexamethasone (CyBorD). Overall response rate was 100 %; four patients (50 %) had complete remissions (CR), two (25 %) had very good partial responses, and two (25 %) had partial responses. Five of six patients (83 %) had organ responses in the heart and/or kidney. A relapsed patient repeatedly achieved CR with the CyBorD treatment. One patient died of sudden cardiac arrest a month after normalization of his serum free light chain level, which may be attributable to his spending the previous 6 months undergoing PBSCT collection and high-dose melphalan with auto-PBSCT. Altogether, the CyBorD regimen achieved high levels of hematological responses relatively quickly (within 2-3 months). The CyBorD regimen, rather than high-dose melphalan treatment, could serve as a first-line therapy for Japanese patients with ALA.

MeSH terms

  • Aged
  • Amyloidosis / diagnosis
  • Amyloidosis / drug therapy*
  • Amyloidosis / metabolism
  • Amyloidosis / mortality
  • Amyloidosis / therapy
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Boronic Acids / administration & dosage
  • Bortezomib
  • Cyclophosphamide / administration & dosage
  • Dexamethasone / administration & dosage
  • Female
  • Humans
  • Immunoglobulin Light Chains*
  • Immunoglobulin Light-chain Amyloidosis
  • Japan
  • Male
  • Middle Aged
  • Monoclonal Gammopathy of Undetermined Significance / drug therapy
  • Multiple Myeloma / drug therapy
  • Pyrazines / administration & dosage
  • Retrospective Studies
  • Survival Analysis
  • Treatment Outcome


  • Boronic Acids
  • Immunoglobulin Light Chains
  • Pyrazines
  • Bortezomib
  • Dexamethasone
  • Cyclophosphamide