Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo, and Department of Molecular Medicine, University of Pavia, Pavia, Italy; and.
National Amyloidosis Centre, University College London Medical School, Royal Free Hospital Campus, London, United Kingdom.
Blood. 2015 Jul 30;126(5):612-5. doi: 10.1182/blood-2015-01-620302. Epub 2015 May 18.
The combination of cyclophosphamide/bortezomib/dexamethasone (CyBorD) showed early promise of high rates of hematologic responses tempered by studies showing the inability to overcome poor prognosis of advanced cardiac amyloidosis. Large studies are needed to clarify its role in light chain (AL) amyloidosis. We report the outcome of 230 patients treated frontline with CyBorD. Overall hematologic response rate was 60%, and in the 201 patients with measurable disease it was 62%, with 43% achieving at least very good partial response (VGPR). Cardiac response was reached in 17% of patients and renal response in 25% of patients. Advanced cardiac stage III patients (amino-terminal pro-natriuretic peptide type B >8500 ng/L) had lower response rates (42%, ≥ VGPR 23%) and poorer survival (median, 7 months). Nevertheless, hematologic response improved survival in these subjects (67% at 2 years), showing the importance of striving for a good response even in this group.
环磷酰胺/硼替佐米/地塞米松（CyBorD）联合方案显示出很高的血液学缓解率的早期希望，但研究表明其无法克服晚期心脏淀粉样变性的不良预后。需要进行大型研究来阐明其在轻链（AL）淀粉样变性中的作用。我们报告了 230 例一线接受 CyBorD 治疗的患者的结果。总体血液学缓解率为 60%，在 201 例可测量疾病患者中为 62%，其中 43%达到至少非常好的部分缓解（VGPR）。17%的患者达到了心脏缓解，25%的患者达到了肾脏缓解。晚期心脏 III 期患者（氨基末端 pro-natriuretic 肽 B 型 >8500ng/L）的缓解率较低（42%，≥VGPR 23%），生存率较差（中位 7 个月）。尽管如此，血液学缓解改善了这些患者的生存（2 年时为 67%），表明即使在这组患者中，也应努力获得良好的缓解。
