Hypereosinophilic Syndrome Subtype Predicts Responsiveness to Glucocorticoids

Hypereosinophilic Syndrome Subtype Predicts Responsiveness to Glucocorticoids
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DOI:
10.1016/j.jaip.2017.06.006
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发表时间:
2018-01-01
影响因子:
9.4
通讯作者:
Klion, Amy D.
Klion, Amy D.
中科院分区:
医学1区
文献类型:
--
作者:
Khoury, Paneez;Abiodun, Annalise O.;Klion, Amy D.

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背景:糖皮质激素(GCs)被认为是血小板衍生生长因子α (PDGFRA)阴性高嗜酸性粒细胞综合征(HESs)的一线治疗方法。尽管如此,对HES中GC反应性的临床预测因素知之甚少。目的:在GC开始前了解GC反应的临床和实验室预测因素,可以更合理地选择HES患者,早期采用二线和替代治疗是合适的。方法:通过对pdgfr阴性HES患者的回顾性图表回顾,根据机构审查委员会批准的方案评估,对GC的反应,定义为绝对嗜酸性粒细胞计数减少到1000/mm以下(3)和症状控制。评估GC检查前获得的人口学、临床和实验室参数以及最终诊断,以确定GC反应的预测因子。比例赔率模型用于单因素和多因素评估预测因子,排列调整P值以校正多重比较。结果:根据GC反应对164例pdgfr阴性HES进行分类。其中,39%的受试者对低剂量(
BACKGROUND: Glucocorticoids (GCs) are considered first-line treatment for platelet-derived growth factor alpha (PDGFRA)-negative hypereosinophilic syndromes (HESs). Despite this, little is known about clinical predictors of GC responsiveness in HES.OBJECTIVE: Knowledge of clinical and laboratory predictors of GC response before initiation of GC could lead to more rational selection of subjects with HES for whom earlier institution of second-line and alternative therapies would be appropriate.METHODS: Response to GC, as defined by the reduction of the absolute eosinophil count to below 1000/mm(3) and control of symptoms, was assessed by a retrospective chart review of subjects with PDGFRA-negative HES evaluated on an institutional review board-approved protocol. Demographic, clinical, and laboratory parameters obtained before institution of GC, as well as final diagnosis, were evaluated to determine predictors of GC response. Proportional odds models were used for univariate and multivariate assessment of predictors with permutation adjusted P values to correct for multiple comparisons.RESULTS: A total of 164 subjects with PDGFRA-negative HES were categorized according to GC response. Of them, 39% of the subjects responded to low dose (