Department of Haematology and Bone Marrow Transplantation, Silesian Medical University, Dabrowski Street 25, 40-032, Katowice, Poland.
Med Oncol. 2012 Jun;29(2):1073-6. doi: 10.1007/s12032-011-9831-1. Epub 2011 Jan 22.
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-PDGFRα-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-PDGFRα-negative HES.
特发性嗜酸性粒细胞增多综合征（HES）由一组异质性疾病组成，其特征为显著的血液嗜酸性粒细胞增多症伴嗜酸性粒细胞相关器官损伤。8 名中位年龄为 42 岁（范围 19-67 岁）的患者因 FIP1L1-PDGFRα 阴性且对既往常规治疗耐药的 HES 接受甲磺酸伊马替尼（IM）治疗。中位既往治疗数为 3 次（范围 2-4 次）。从诊断到开始 IM 治疗的中位时间为 112 个月（范围 2-293 个月）。4 名患者每日接受 100mg IM 治疗，而其余 4 名患者每日接受 400mg IM 治疗。4 名男性患者（50%）使用 100mg 每日 IM（n=2）和 400mg（n=2）后，中位时间为 7 天（范围 3-150 天）达到完全血液学缓解（CHR）。IM 应答者的 IM 中位治疗时间为 18 个月（范围 2-88 个月）。在整个治疗过程中未报告不良反应。2 名患者在接受 100mg 每周 IM 治疗时维持 CHR。4 名患者（2 名男性和 2 名女性）IM 治疗失败。非应答患者的 IM 中位治疗时间为 3 周（范围 3-12 周）。与 IM 应答者相比，非应答 HES 患者年龄较大，血液嗜酸性粒细胞百分比较低。我们的结果表明，即使使用低于常规剂量的 IM，也可能诱导并维持 FIP1L1-PDGFRα 阴性 HES 的长期缓解。
