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. © 2012 by The American Society of Hematology.
CITATION STYLE
Venner, C. P., Lane, T., Foard, D., Rannigan, L., Gibbs, S. D. J., Pinney, J. H., … Wechalekar, A. D. (2012). Cyclophosphamide, bortezomib,anddexamethasone therapy in AL amyloidosis is associated with high clonal response rates and prolonged progression-free survival. Blood, 119(19), 4387–4390. https://doi.org/10.1182/blood-2011-10-388462
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