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伊马替尼治疗FIP1L1-PDGFRA阳性高嗜酸性粒细胞综合征的疗效

[Efficacy of imatinib in FIP1L1-PDGFRA positive hypereosinophilic syndrome].

作者信息

Menif S, Ben Romdhane N, Hafsia R

机构信息

Laboratoire d'hématologie moléculaire et cellulaire, institut Pasteur, Tunis, Tunisia.

出版信息

Rev Med Interne. 2008 Jan;29(1):65-7. doi: 10.1016/j.revmed.2007.09.036. Epub 2007 Oct 25.

Abstract

Hypereosinophilic syndromes (HES) are a heterogeneous group of disorders characterized by marked peripheral blood and tissue eosinophilia resulting in organ damage. Recent advances in molecular biology have led to the identification of a FIP1L1-PDGFRA fusion gene as a recurrent abnormality in some patients with HES. This fusion gene results from a cryptic 4q12 interstitial deletion involving an 800 kb region. Recent reports indicate that this subtype of HES is imatinib responsive with rapid and complete haematological remissions. Here we report two patients successfully treated with imatinib.

摘要

高嗜酸性粒细胞综合征(HES)是一组异质性疾病,其特征为外周血和组织嗜酸性粒细胞显著增多,进而导致器官损害。分子生物学的最新进展已使FIP1L1-PDGFRA融合基因被鉴定为某些HES患者中反复出现的异常。该融合基因由涉及800 kb区域的隐匿性4q12间质性缺失产生。最近的报告表明,这种HES亚型对伊马替尼有反应,可实现快速且完全的血液学缓解。在此,我们报告两名成功接受伊马替尼治疗的患者。

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