Division of Hematology, Mayo Clinic, Rochester, MN, USA.
Bone Marrow Transplant. 2011 Jul;46(7):970-5. doi: 10.1038/bmt.2010.234. Epub 2010 Oct 11.
The role of auto-SCT in the management of Ig light-chain (AL) amyloidosis remains undefined. In this study, we report 422 patients who received auto-SCT for AL amyloidosis to compare outcomes of patients treated before January 2006 with those treated from January 2006 to 1 December 2009. Day 100 all-cause mortality decreased over this time period from 12 to 7% (P=0.09). Survival at 2 years increased from 78 to 82%. The major determinants of early mortality (before day 100) were the presence of cardiac involvement by amyloid with increased levels of cardiac biomarkers, lower serum albumin, higher serum creatinine and a higher number of organs involved. On multivariate survival analysis, higher levels of serum troponin T and N-terminal pro-brain natriuretic peptide were the only predictors of early mortality after auto-SCT. Improved supportive care and refined patient selection have improved the safety margin of patients undergoing auto-SCT; short-term mortality showed a more than 40% decrease after 2005.
自体造血干细胞移植（auto-SCT）在免疫球蛋白轻链（AL）淀粉样变性治疗中的作用尚不清楚。本研究报道了 422 例接受 auto-SCT 治疗的 AL 淀粉样变性患者，旨在比较 2006 年 1 月前治疗患者与 2006 年 1 月至 2009 年 12 月 1 日治疗患者的结局。在此期间，第 100 天全因死亡率从 12%降至 7%（P=0.09）。2 年生存率从 78%增至 82%。早期死亡率（第 100 天前）的主要决定因素是淀粉样物质引起的心脏受累，且伴有心脏标志物水平升高、血清白蛋白降低、血清肌酐升高和受累器官数量增加。多因素生存分析显示，血清肌钙蛋白 T 和 N 末端脑利钠肽前体水平升高是 auto-SCT 后早期死亡的唯一预测因素。支持治疗的改进和患者选择的细化提高了接受 auto-SCT 治疗患者的安全性；2005 年后，短期死亡率下降了 40%以上。
