The outcomes in systemic AL amyloidosis are dependent on the depth of haematologic response. However, there is limited data on the impact of the speed of response on outcomes. Here we report the impact of speed of response in a cohort of AL patients treated with upfront Bortezomib. Patients seen from February 2010 until August 2019 are included in the present analysis. 1194 & 1133 patients comprised the ITT and 1-month landmark cohorts. In the landmark cohort, 137 (11.5%), 270 (22.6%), 252 (21.1%) and 352 (31.1%) patients had a CR, VGPR, PR and NR at 1-month. Patients with ≥ VGPR at 1-month had significantly better survival (median not reached; at the end of 1, 2, 5,10 years, 87%/92%, 83%/87%, 68%/72% and 63%/58% of patients in CR/VGPR, respectively, were alive) compared to those with a PR (median OS 60 months) or NR (median OS 32 months) (p < 0.005). At 1-month, patients with CR and iFLC < 20 mg/l had a significantly better survival compared to CR and iFLC > 20 mg/l (p = 0.005). Reaching ≥ VGPR at 1-month significantly improved survival in all Mayo disease stages. In conclusion, patients achieving an early deep haematologic response have a significantly superior survival irrespective of cardiac involvement.

系统性 AL 淀粉样变性的结果取决于血液学反应的深度。然而，关于响应速度对结果的影响的数据有限。在这里，我们报告了接受预先硼替佐米治疗的 AL 患者队列中反应速度的影响。2010 年 2 月至 2019 年 8 月期间就诊的患者包括在本分析中。1194 和 1133 名患者包括 ITT 和 1 个月的标志性队列。在具有里程碑意义的队列中，137 (11.5%)、270 (22.6%)、252 (21.1%) 和 352 (31.1%) 名患者在 1 个月时达到 CR、VGPR、PR 和 NR。1 个月时 VGPR ≥ 的患者生存率显着提高（未达到中位数；在 1、2、5、10 年末，分别为 87%/92%、83%/87%、68%/72% 和 63% /58% 的 CR/VGPR 患者分别存活）与 PR（中位 OS 60 个月）或 NR（中位 OS 32 个月）的患者相比（p  < 0.005)。在 1 个月时，与 CR 和 iFLC > 20 mg/l 相比，CR 和 iFLC < 20 mg/l 的患者的生存率显着提高 ( p  = 0.005)。在 1 个月时达到 ≥ VGPR 可显着提高所有梅奥病阶段的生存率。总之，无论心脏受累如何，实现早期深部血液学反应的患者都有显着优越的生存率。