Imatinib mesylate may induce long-term clinical response in FIP1L1-PDGFRα-negative hypereosinophilic syndrome
Imatinib mesylate may induce long-term clinical response in FIP1L1-PDGFRα-negative hypereosinophilic syndrome
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DOI：
10.1007/s12032-011-9831-1
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发表时间：
2012-06-01
期刊：
影响因子：
3.4
通讯作者：
Kyrcz-Krzemien, Slawomira
中科院分区：
文献类型：
--
作者：
Helbig, Grzegorz;Hus, Marek;Kyrcz-Krzemien, Slawomira
特发性嗜酸性粒细胞增多综合征（HES）包括一组异质性疾病，其特征是显著的血液嗜酸性粒细胞增多伴嗜酸性粒细胞相关的器官损害。8例诊断时中位年龄为42岁（范围19-67）的患者接受了甲磺酸伊马替尼（IM）治疗对既往常规治疗耐药的FIP 1 L1-PDGFR α阴性HES。既往治疗的中位次数为3次（范围2-4次）。从诊断到开始IM的中位时间为112个月（范围2-293）。4例患者每日接受100 mg IM治疗，而其余4例患者每日接受400 mg IM治疗。4例男性患者（50%）在接受100 mg每日IM（n = 2）和400 mg每日IM（n = 2）治疗后（中位时间7天，范围3-150天）达到完全血液学缓解（CR）。IM应答者的IM治疗中位持续时间为18个月（范围2-88）。整个治疗期间未报告不良事件。2例患者在每周IM 100 mg时维持治疗。4例患者（2例男性和2例女性）IM治疗失败。无应答患者IM的中位持续时间为3周（范围3-12）。与IM应答者相比，无应答的HES患者年龄显著更大，血嗜酸性粒细胞增多百分比更低。我们的研究结果表明，IM，即使在低于常规剂量，可以诱导和维持FIP 1 L1-PDGFR α阴性HES的长期缓解。
The idiopathic hypereosinophilic syndrome (HES) comprises a heterogenous group of disorders characterized by marked blood eosinophilia with eosinophilia-associated organ damage. Eight patients with a median age at diagnosis of 42 years (range 19-67) received imatinib mesylate (IM) for FIP1L1-PDGFR alpha-negative HES resistant to previous conventional treatment. Median number of prior therapies was 3 (range 2-4). Median time from diagnosis to IM initiation was 112 months (range 2-293). Four patients were treated daily with 100 mg IM, whereas the remaining four patients were treated daily with 400 mg IM. Four male patients (50%) achieved complete haematologic response (CHR) after median of 7 days (range 3-150) using 100 mg daily IM (n = 2) and 400 mg (n = 2). Median duration of IM treatment for IM responders was 18 months (range 2-88). No adverse events were reported throughout the treatment duration. Two patients maintained CHR while on 100 mg weekly IM. Four patients (2 men and 2 women) failed IM treatment. Median duration of IM for non-responding patients was 3 weeks (range 3-12). Non-responding HES patients were significantly older and had lower percentage of blood eosinophilia when compared with IM responders. Our results suggest that IM, even at lower than conventional doses, may induce and maintain long-term remission for FIP1L1-PDGFR alpha-negative HES.