Cyclophosphamide, bortezomib, and dexamethasone therapy in AL amyloidosis is associated with high clonal response rates and prolonged progression-free survival

Blood. 2012 May 10;119(19):4387-90. doi: 10.1182/blood-2011-10-388462. Epub 2012 Feb 13.

Abstract

Bortezomib has shown great promise in the treatment of amyloid light-chain (AL) amyloidosis. We present our experience of 43 patients with AL amyloidosis who received cyclophosphamide, bortezomib, and dexamethasone (CVD) upfront or at relapse. Of these, 74% had cardiac involvement and 46% were Mayo Cardiac Stage III. The overall hematologic response rate was 81.4%, including complete response (CR) in 41.9% and very good partial response with >90% decrease in difference between involved/uninvolved light chain (VGPR-dFLC) in 51.4%. Patients treated upfront had higher rates of CR (65.0%) and VGPR-dFLC (66.7%). The estimated 2-year progression-free survival was 66.5% for patients treated upfront and 41.4% for relapsed patients. Those attaining a CR or VGPR-dFLC had a significantly better progression-free survival (P=.002 and P=.026, respectively). The estimated 2-year overall survival was 97.7% (94.4% in Mayo Stage III patients). CVD is a highly effective regimen producing durable responses in AL amyloidosis; the deep clonal responses may overcome poor prognosis in advanced-stage disease.

Publication types

  • Evaluation Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyloid / metabolism
  • Amyloidosis / drug therapy*
  • Amyloidosis / metabolism
  • Antineoplastic Combined Chemotherapy Protocols / adverse effects
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Boronic Acids / administration & dosage*
  • Boronic Acids / adverse effects
  • Bortezomib
  • Clonal Evolution / drug effects*
  • Cohort Studies
  • Cyclophosphamide / administration & dosage*
  • Cyclophosphamide / adverse effects
  • Dexamethasone / administration & dosage*
  • Dexamethasone / adverse effects
  • Disease-Free Survival
  • Female
  • Humans
  • Male
  • Middle Aged
  • Pyrazines / administration & dosage*
  • Pyrazines / adverse effects
  • Recurrence
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Amyloid
  • Boronic Acids
  • Pyrazines
  • Bortezomib
  • Dexamethasone
  • Cyclophosphamide