Light chain type predicts organ involvement and survival in AL amyloidosis patients receiving stem cell transplantation

轻链类型可预测接受干细胞移植的AL淀粉样变性患者的器官受累情况和生存率。

阅读:1

Abstract

We evaluated the impact of light chain type, lambda (λ) or kappa (κ), on disease features and outcomes in patients with immunoglobulin light chain (AL) amyloidosis receiving stem cell transplant at the Mayo Clinic between October 2002 and August 2016. Patients with λ AL amyloidosis had higher rates of renal and neurological involvement (λ 69% vs κ 57%, P = .02 and λ 16% vs κ 9%, P = .03, respectively). Patients with κ AL amyloidosis had more hepatic involvement (λ 7% vs κ 18%, P = .0003). Complete response rate was 43% for both groups and overall response rates were similar (λ 85% vs κ 91%, P = .12). Patients with κ light chain amyloidosis had better progression-free and overall survival (PFS: λ 74 months vs κ 101 months, P = .0064 and OS: λ 121 months vs κ not reached, P = .003). Mayo stage 2004 was more predictive of survival in the λ cohort (median OS of 143 months stage I vs 77 months stage II vs 33 months stage III, P < .0001) than in the κ cohort (median OS not reached for stage I and II and 102 months for stage III, P = .044). Conditioning dose predicted survival in the λ cohort only (median OS 149 months for melphalan 200 mg/m(2) vs 50 months for melphalan <200 mg/m(2), P < .0001; median OS κ not reached for melphalan 200 mg/m(2) or <200 mg/m(2), P = .38). On multivariate analysis, light chain type remained an independent predictor of survival. Light chain type predicts organ involvement and survival in patients with AL amyloidosis receiving stem cell transplant.

特别声明

1、本页面内容包含部分的内容是基于公开信息的合理引用;引用内容仅为补充信息,不代表本站立场。

2、若认为本页面引用内容涉及侵权,请及时与本站联系,我们将第一时间处理。

3、其他媒体/个人如需使用本页面原创内容,需注明“来源:[生知库]”并获得授权;使用引用内容的,需自行联系原作者获得许可。

4、投稿及合作请联系:info@biocloudy.com。