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对于新诊断的轻链淀粉样变性患者,硼替佐米治疗后游离轻链(dFLC)小于 10mg/L 预测器官反应更好,且下一次治疗的时间更长。

Posttreatment dFLC less than 10 mg/L predicts superior organ response and longer time to next treatment in newly diagnosed light-chain amyloidosis patients treated with bortezomib.

机构信息

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.

Department of Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.

出版信息

Leuk Lymphoma. 2021 Apr;62(4):874-882. doi: 10.1080/10428194.2020.1849675. Epub 2020 Nov 20.

Abstract

Recently, a difference between involved and uninvolved free light chains (dFLC) less than 10 mg/L after treatment (stringent dFLC response) was reported to be associated with superior survival in light-chain (AL) amyloidosis. We conducted a retrospective study of AL amyloidosis patients treated with bortezomib to investigate the predictive value of a stringent dFLC response. Two hundred and thirty-five patients were included. The cardiac and renal responses were much higher in patients achieving a stringent dFLC response (86.5% versus 42.7% and 75.9% versus 38.2%,  < .001). Patients with a stringent dFLC response had significantly longer overall survival and time to next treatment (TNT). Among the very good partial response (VGPR) patients, the TNT of stringent dFLC responders was superior to those of the remaining VGPR patients ( = .045) and comparable to those of complete response patients. In conclusion, a stringent dFLC response might be added to current response criteria for AL amyloidosis.

摘要

最近,有研究报道,在治疗后游离轻链差值(dFLC)小于 10mg/L(严格 dFLC 反应)与轻链(AL)淀粉样变性患者的生存改善相关。我们对接受硼替佐米治疗的 AL 淀粉样变性患者进行了一项回顾性研究,以探讨严格 dFLC 反应的预测价值。共纳入 235 例患者。达到严格 dFLC 反应的患者在心脏和肾脏方面的反应率更高(86.5%比 42.7%和 75.9%比 38.2%,<0.001)。达到严格 dFLC 反应的患者总生存时间和至下次治疗时间(TNT)显著更长。在非常好的部分缓解(VGPR)患者中,严格 dFLC 反应者的 TNT 优于其余 VGPR 患者(=0.045),与完全缓解患者相当。总之,严格 dFLC 反应可作为 AL 淀粉样变性的现有缓解标准的补充。

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