伊马替尼治疗多浆膜炎伴FIP1L1-PDGFRA阴性高嗜酸性粒细胞综合征的疗效
[Efficiency of imatinib in polyserositis revealing a FIP1L1-PDGFRA-negative hypereosinophilic syndrome].
作者信息
Green L, Kahn J-E, Dufour J-F, Le Scanff J, Charhon A, Broussolle C, Sève P
机构信息
Service de médecine interne, hôtel Dieu, 1, place de l'Hôpital, Lyon cedex, France.
出版信息
Rev Med Interne. 2010 Apr;31(4):305-8. doi: 10.1016/j.revmed.2009.08.005. Epub 2010 Feb 18.
INTRODUCTION
Hypereosinophilic syndromes rarely manifest as polyserositis. Imatinib mesylate is the reference treatment for myeloid variants of FIP1L1-PDGFRA-positive hypereosinophilic syndromes. A response to imatinib has also been reported in FIP1L1-PDGFRA-negative hypereosinophilic syndromes.
CASE REPORT
We report a 25-year-old man who presented with a FIP1L1-PDGFRA-negative hypereosinophilic syndrome, with severe pericardial effusion and bilateral pleuritis. Imatinib mesylate at the dose of 100mg daily was started because of high-dose corticosteroids dependence. A response was noted after 15 days of treatment. Corticosteroids were discontinued after 7 months and the patient remained asymptomatic after 23 months of treatment.
CONCLUSION
This report evidences the association of polyserositis with hypereosinophilic syndromes and the potential efficacy of imatinib mesylate even in FIP1L1-PDGFRA-negative patients.
引言
嗜酸性粒细胞增多综合征很少表现为多浆膜炎。甲磺酸伊马替尼是FIP1L1-PDGFRA阳性嗜酸性粒细胞增多综合征髓系变异型的标准治疗药物。在FIP1L1-PDGFRA阴性嗜酸性粒细胞增多综合征中也有对甲磺酸伊马替尼有反应的报道。
病例报告
我们报告一名25岁男性，患有FIP1L1-PDGFRA阴性嗜酸性粒细胞增多综合征，伴有严重心包积液和双侧胸膜炎。由于对高剂量皮质类固醇依赖，开始每日服用100mg甲磺酸伊马替尼。治疗15天后观察到有反应。7个月后停用皮质类固醇，治疗23个月后患者仍无症状。
结论
本报告证明了多浆膜炎与嗜酸性粒细胞增多综合征的关联以及甲磺酸伊马替尼即使在FIP1L1-PDGFRA阴性患者中的潜在疗效。
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