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Efficacy of Bortezomib, Cyclophosphamide and Dexamethasone in treatment of naive patients with high risk cardiac AL amyloidosis (Mayo Clinic stage III).
Haematologica. 2014 May 23. pii: haematol.2014.104109. [Epub ahead of print] doi:
Jaccard A, Comenzo RL, Hari P, Hawkins PN, Roussel M, Morel P, Macro M, Pellegrin JL, Lazaro E, Mohty D, Mercie P, Decaux O, Gillmore J, Lavergne D, Bridoux F, Wechalekar AD, Venner CP
Abstract:
.....Here we present an international, multi-centre series of 60 patients with Mayo Clinic stage III cardiac amyloidosis to assess the impact of this regimen in improving outcomes in this poor risk group. Median follow-up for the entire cohort is 11.8 months. The overall response rate was 68%.
In a landmark analysis, examining patients who survived greater than 3 months, the overall response rate was 86%. A cardiac response was seen in 32 % of patients. Estimated 1-year survival for the whole cohort is 57%, and 24 patients (40%) died while on therapy
. Although unable to save the poorest risk patients, this combination can achieve a high number of hematologic and cardiac responses, likely improving overall survival and justifying a prospective trial.
In a landmark analysis, examining patients who survived greater than 3 months, the overall response rate was 86%. A cardiac response was seen in 32 % of patients. Estimated 1-year survival for the whole cohort is 57%, and 24 patients (40%) died while on therapy
. Although unable to save the poorest risk patients, this combination can achieve a high number of hematologic and cardiac responses, likely improving overall survival and justifying a prospective trial.