Improved survival outcomes with anakinra over etoposide-based therapies for the management of adults with hemophagocytic lymphohistiocytosis: a retrospective multicenter research network study

IF 3.4 3区医学 Q2 HEMATOLOGY Therapeutic Advances in Hematology Pub Date : 2024-04-17 DOI:10.1177/20406207241245517

Benjamin J. Lee

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Abstract

Background:Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening, hyperinflammatory syndrome for which etoposide-based regimens have historically been the standard of care. Recent reports have described positive outcomes with the utilization of ruxolitinib or anakinra although these studies are often limited to small samples.Objectives:We aimed to compare the efficacy of ruxolitinib, anakinra, and etoposide-based therapies for the management of HLH in adult patients.Design:We performed a population-based, multicenter, retrospective cohort study utilizing the TriNetX Networks database.Methods:Adult patients (⩾18 years) diagnosed with HLH who received first-line treatment with ruxolitinib, anakinra, or etoposide between 2008 and 2023 were analyzed. The primary endpoint was overall survival (OS) at 1 year. A 1:1 propensity-score matching analysis was utilized.Results:Anakinra ( p = 0.020) but not ruxolitinib ( p = 0.19) resulted in a significantly higher 1-year OS when compared with etoposide-based therapies.Conclusions:Anakinra is effective for the management of adult patients with HLH.

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在治疗成人嗜血细胞淋巴组织细胞增多症时，阿纳金拉比依托泊苷疗法的生存率更高：一项回顾性多中心研究网络研究

背景：嗜血细胞淋巴组织细胞增多症（HLH）是一种罕见的危及生命的高炎症综合征，以依托泊苷为基础的治疗方案历来是治疗的标准。最近有报道称，尽管这些研究往往局限于小样本，但使用鲁索利替尼或阿纳喹来治疗HLH取得了积极的疗效。目的：我们旨在比较鲁索利替尼、阿纳喹来和依托泊苷为基础的疗法治疗成人HLH的疗效。设计：我们利用 TriNetX Networks 数据库开展了一项基于人群的多中心回顾性队列研究。方法：我们分析了 2008 年至 2023 年间确诊为 HLH 的成年患者（⩾18 岁）接受 ruxolitinib、anakinra 或依托泊苷一线治疗的情况。主要终点是1年的总生存期（OS）。结果：与依托泊苷疗法相比，Anakinra（p = 0.020）的1年OS显著高于ruxolitinib（p = 0.19）。

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来源期刊

Therapeutic Advances in Hematology HEMATOLOGY-

CiteScore

4.30

自引率

0.00%

发文量

审稿时长

7 weeks

期刊介绍： Therapeutic Advances in Hematology delivers the highest quality peer-reviewed articles, reviews, and scholarly comment on pioneering efforts and innovative studies across all areas of hematology. The journal has a strong clinical and pharmacological focus and is aimed at clinicians and researchers in hematology, providing a forum in print and online for publishing the highest quality articles in this area.