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

Christopher P Venner, Thirusha Lane, Darren Foard, Lisa Rannigan, Simon DJ Gibbs, Jennifer H Pinney, Carol J Whelan, Helen J Lachmann, Julian D Gillmore, Philip N Hawkins and Ashutosh D Wechalekar


Bortezomib has shown great promise in the treatment of AL amyloidosis. We present our experience of 43 patients with AL amyloidosis who received cyclophosphamide, bortezomib and dexamethasone (CVD) upfront or at relapse. 74% had cardiac involvement and 46% were Mayo cardiac stage III. Overall hematologic response rate was 81.4%, including CR in 41.9% and VGPR-dFLC (>90% decrease in difference between involved/uninvolved light chain) in 51.4%. Patients treated upfront had higher rates of CR (65.0%) and VGPR-dFLC (66.7%). The estimated 2-year PFS was 66.5% for patients treated upfront and 41.4% for relapsed patients. Those attaining a CR or VGPR-dFLC had a significantly better PFS (P = 0.002 and P = 0.026, respectively). The estimated 2-year overall survival is 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.

  • Submitted October 28, 2011.
  • Accepted January 20, 2012.