Imatinib is effective in some PDGFRA/B-negative hypereosinophilic syndromes: A step closer to unveiling underlying mechanisms.

IF 5.1 2区 医学 Q1 HEMATOLOGY British Journal of Haematology Pub Date : 2024-10-28 DOI:10.1111/bjh.19853
Giuseppe G Loscocco, Grzegorz Helbig
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引用次数: 0

Abstract

Hypereosinophilic syndromes (HES) comprise different clonal, reactive, or idiopathic disorders characterized by elevated eosinophil levels and subsequent organ damage. Kim et al. in a multicentre, single-arm, prospective phase II study, treated 32 patients with PDGFRA/B-negative HES with imatinib at the dose of 100-400 mg daily. Respective overall and complete haematological response rates were 46.9% and 18.8%, and the median time to response was 1.5 months. The molecular basis of responses was identified by using whole-exome and whole-transcriptome sequencing in 11 patients. STAT5B::RARA, PAK2::PIGX, and FIP1L1::CHIC2 fusions were identified in responders, whereas RNF130::BRAF and WNK1::KDM5A were identified in non-responders. Imatinib could be a therapeutic option for some, possibly clonal, PDGFRA/B-negative HES. Commentary on: Kim et al. Phase II trial of imatinib mesylate in patients with PDGFRA/B-negative hypereosinophilic syndrome. Br J Haematol 2024 (Online ahead of print). doi: 10.1111/bjh.19828.

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伊马替尼对某些PDGFRA/B阴性的高嗜酸性粒细胞综合征有效:向揭示潜在机制又迈进了一步。
嗜酸性粒细胞过多综合征(HES)由不同的克隆性、反应性或特发性疾病组成,其特点是嗜酸性粒细胞水平升高和随后的器官损伤。Kim 等人在一项多中心、单臂、前瞻性 II 期研究中,用伊马替尼治疗了 32 例 PDGFRA/B 阴性 HES 患者,剂量为每天 100-400 毫克。总反应率和完全血液学反应率分别为46.9%和18.8%,中位反应时间为1.5个月。通过对11名患者进行全外显子组和全转录组测序,确定了应答的分子基础。在应答者中发现了STAT5B::RARA、PAK2::PIGX和FIP1L1::CHIC2融合,而在非应答者中发现了RNF130::BRAF和WNK1::KDM5A。对于某些可能是克隆性的PDGFRA/B阴性HES,伊马替尼可能是一种治疗选择。相关评论Kim等:甲磺酸伊马替尼治疗PDGFRA/B阴性高嗜酸性粒细胞综合征患者的II期试验。Doi: 10.1111/bjh.19828.
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
8.60
自引率
4.60%
发文量
565
审稿时长
1 months
期刊介绍: The British Journal of Haematology publishes original research papers in clinical, laboratory and experimental haematology. The Journal also features annotations, reviews, short reports, images in haematology and Letters to the Editor.
期刊最新文献
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