Early minimal residual disease eradication in light chain amyloidosis generates deeper and faster cardiac response.

IF 3.2 4区 医学 Q2 MEDICINE, RESEARCH & EXPERIMENTAL
Tianhong Xu, Jing Li, Yang Yang, Wenjing Wang, Chi Zhou, Pu Wang, Chenqi Yu, Peng Liu
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Abstract

Minimal residual disease (MRD) is of growing interest in light chain (AL) amyloidosis and is associated with higher rates of cardiac response. A new graded cardiac response criteria has been proposed for better assessment of cardiac improvement. We evaluated MRD status in 63 patients with cardiac AL amyloidosis using next generation flow cytometry (sensitivity ≥ 1*10-5) within four cycles after treatment initiation and cardiac response kinetics. All patients were treated with first-line proteasome inhibitor (100%) and predominantly bortezomib (87.3%). The overall early MRD negative rates were 33.3%. Patients who achieved early MRD negativity were less likely to harbor t(11;14) (21.1% vs 57.5%, P = 0.009). The MRD negative rates amongst patients in hematologic complete response were 66.7% (14/21), and in very good partial response 29.2% (7/24). Early MRD negativity was associated with a higher likelihood of achieving ≥ cardiac partial response (≥ CarPR) (66.7% vs 38.1%, P = 0.032) and ≥ cardiac very good partial response (≥ CarVGPR) (38.1% vs 11.9%, P = 0.023) throughout first-line therapy. The cumulative incidence curve of achieving ≥ CarPR (P = 0.034) and ≥ CarVGPR (P = 0.026) showed significant difference between early MRD negative and positive group. After a median follow-up time of 27.2 months, the median progression free survival was longer in early MRD negative group (not reached vs 31.3 months, P = 0.033). Early MRD eradication in cardiac AL amyloidosis generated deeper and faster cardiac organ response.

早期根除轻链淀粉样变性的极小残留病变可产生更深更快的心脏反应。
在轻链(AL)淀粉样变性中,最小残留病(MRD)越来越受到关注,它与较高的心脏反应率有关。为了更好地评估心脏疾病的改善情况,人们提出了一种新的心脏反应分级标准。我们采用新一代流式细胞术(灵敏度≥ 1*10-5)评估了63例心脏AL淀粉样变性患者在治疗开始后四个周期内的MRD状态和心脏反应动力学。所有患者都接受了一线蛋白酶体抑制剂治疗(100%),主要是硼替佐米(87.3%)。总体早期MRD阴性率为33.3%。早期MRD阴性的患者携带t(11;14)的可能性较低(21.1% vs 57.5%,P = 0.009)。血液学完全反应患者的MRD阴性率为66.7%(14/21),部分反应非常好的患者为29.2%(7/24)。在整个一线治疗过程中,早期MRD阴性与更有可能实现≥心脏部分反应(≥ CarPR)(66.7% vs 38.1%,P = 0.032)和≥心脏非常好部分反应(≥ CarVGPR)(38.1% vs 11.9%,P = 0.023)相关。早期MRD阴性组和阳性组达到≥CarPR(P = 0.034)和≥CarVGPR(P = 0.026)的累积发生率曲线显示出显著差异。中位随访时间为 27.2 个月后,早期 MRD 阴性组的中位无进展生存期更长(未达到 vs 31.3 个月,P = 0.033)。在心脏AL淀粉样变性病中早期根除MRD可产生更深更快的心脏器官反应。
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来源期刊
Clinical and Experimental Medicine
Clinical and Experimental Medicine 医学-医学:研究与实验
CiteScore
4.80
自引率
2.20%
发文量
159
审稿时长
2.5 months
期刊介绍: Clinical and Experimental Medicine (CEM) is a multidisciplinary journal that aims to be a forum of scientific excellence and information exchange in relation to the basic and clinical features of the following fields: hematology, onco-hematology, oncology, virology, immunology, and rheumatology. The journal publishes reviews and editorials, experimental and preclinical studies, translational research, prospectively designed clinical trials, and epidemiological studies. Papers containing new clinical or experimental data that are likely to contribute to changes in clinical practice or the way in which a disease is thought about will be given priority due to their immediate importance. Case reports will be accepted on an exceptional basis only, and their submission is discouraged. The major criteria for publication are clarity, scientific soundness, and advances in knowledge. In compliance with the overwhelmingly prevailing request by the international scientific community, and with respect for eco-compatibility issues, CEM is now published exclusively online.
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