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轻链淀粉样变性的治疗模式和结局:阿根廷淀粉样变性报告中的机构登记。

Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina.

机构信息

Internal Medicine Department, Internal Medicine Research Unit, CONICET, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

Internal Medicine Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

出版信息

PLoS One. 2022 Oct 27;17(10):e0274578. doi: 10.1371/journal.pone.0274578. eCollection 2022.

Abstract

Light chain (AL) amyloidosis is a form of systemic amyloidosis, causing organ dysfunction, mainly affecting the heart and kidney. Patient-tailored and risk-adapted decision making is critical in AL amyloidosis management. There is limited real-world evidence data from Argentina and Latin America regarding the treatment approaches for AL amyloidosis. This retrospective cohort study aimed to describe the treatment patterns and outcomes in adult patients (>18 years) diagnosed with AL amyloidosis at the Hospital Italiano in Buenos Aires, Argentina, using a 10-yearfollow-up data (June 1, 2010 to May 31, 2019) from the institutional registry of amyloidosis (IRA). The study population had a mean age of 63 years and 54.4% weremale. Heart and kidney were the most frequently affected organs. Of the 90 eligible patients included in the study, 70underwent treatment. Bortezomib-based regimen was the preferred first-line treatment (75.7% patients). Overall,54.4% of the patients presented a deep response (complete or very good partial response). Median overall survival (OS) was 5years, the 1-year OS and progression free survival rates were 80% (95% confidence interval [CI]: 68-87) and 80% (95%CI 68-87)), respectively. This study provides vital real-world evidence for the long-term treatment patterns and survival in a large cohort of AL amyloidosis patients in Argentina.

摘要

轻链(AL)淀粉样变性是一种系统性淀粉样变性,可导致器官功能障碍,主要影响心脏和肾脏。针对 AL 淀粉样变性患者进行个体化和风险适应的决策至关重要。阿根廷和拉丁美洲关于 AL 淀粉样变性治疗方法的真实世界证据数据有限。本回顾性队列研究旨在使用来自阿根廷布宜诺斯艾利斯的意大利医院(Hospital Italiano)淀粉样变性机构登记处(IRA)的 10 年随访数据(2010 年 6 月 1 日至 2019 年 5 月 31 日),描述 90 名在该医院确诊的 AL 淀粉样变性成年患者(>18 岁)的治疗模式和结局。研究人群的平均年龄为 63 岁,54.4%为男性。心脏和肾脏是最常受影响的器官。在纳入研究的 90 名符合条件的患者中,有 70 名接受了治疗。硼替佐米为基础的方案是首选的一线治疗方案(75.7%的患者)。总体而言,54.4%的患者表现出深度缓解(完全缓解或非常好的部分缓解)。中位总生存期(OS)为 5 年,1 年 OS 和无进展生存期率分别为 80%(95%CI:68-87)和 80%(95%CI 68-87)。本研究为阿根廷大型 AL 淀粉样变性患者的长期治疗模式和生存情况提供了重要的真实世界证据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf61/9612475/13c477940d07/pone.0274578.g001.jpg

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