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関連論文:
img  1:  Cyclophosphamide, bortezomib, and dexamethasone therapy in AL amyloidosis is associated with high clonal response rates and prolonged progression-free survival.
 
著者: Christopher P Venner, Thirusha Lane, Darren Foard, Lisa Rannigan, Simon D J Gibbs, Jennifer H Pinney, Carol J Whelan, Helen J Lachmann, Julian D Gillmore, Philip N Hawkins, Ashutosh D Wechalekar
雑誌名: Blood. 2012 May 10;119(19):4387-90. doi: 10.1182/blood-2011-10-388462. Epub 2012 Feb 13.
Abstract/Text 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.

PMID 22331187  Blood. 2012 May 10;119(19):4387-90. doi: 10.1182/blood-2011-10-388462. Epub 2012 Feb 13.
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