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嗜酸性粒细胞增多症的诊治进展。

Advances in the diagnosis and treatment of eosinophilia.

机构信息

Department of Hematology and Bone Marrow Transplantation, Silesian Medical University, Katowice, Poland.

出版信息

Curr Opin Hematol. 2014 Jan;21(1):3-7. doi: 10.1097/MOH.0000000000000011.

Abstract

PURPOSE OF REVIEW

Eosinophils play an important role in the pathogenesis of allergic, infectious and malignant diseases. Over the last decade, new diagnostic tools and treatment modalities have led to the re-evaluation of the existing definition of eosinophilic disorders. This review discusses a recent proposal for new terminology and classification of hypereosinophilia. The results of targeted therapy for hypereosinophilia-related disorders are also summarized.

RECENT FINDINGS

A panel of multidisciplinary experts agreed on unifying definitions and criteria of eosinophilia-associated disorders and created a new classification of hypereosinophilia-related conditions based on clinical, haematological and laboratory findings as well as the underlying cause of hypereosinophilia. Recent results of the treatment of idiopathic hypereosinophilic syndrome (HES) with the anti-interleukin 5 monoclonal antibody mepolizumab showed its efficacy and manageable safety profile. The treatment of platelet-derived growth factor alpha (PDGFRA)-positive HES with imatinib demonstrated long-lasting efficacy and low likelihood of drug resistance.

SUMMARY

The unifying terminology and definitions should aid physicians caring for patients with hypereosinophilia. Despite much progress, serum biomarkers correlate with disease severity and predict responses to treatment that are needed. There is also a great need for understanding and specific therapy for PDGFRA-negative HES.

摘要

综述目的:嗜酸性粒细胞在过敏、感染和恶性疾病的发病机制中起着重要作用。在过去十年中,新的诊断工具和治疗方法促使人们重新评估现有的嗜酸性粒细胞疾病定义。本文讨论了嗜酸性粒细胞增多症的新术语和分类的最新建议。还总结了嗜酸性粒细胞增多相关疾病的靶向治疗结果。

最新发现:一组多学科专家就嗜酸性粒细胞相关疾病的统一定义和标准达成一致,并根据临床、血液学和实验室发现以及嗜酸性粒细胞增多的潜在原因,对嗜酸性粒细胞增多相关疾病进行了新的分类。最近使用抗白细胞介素 5 单克隆抗体美泊利珠单抗治疗特发性嗜酸性粒细胞综合征 (HES) 的结果表明其疗效和可管理的安全性。伊马替尼治疗血小板衍生生长因子受体 α (PDGFRA) 阳性 HES 显示出持久的疗效和较低的耐药可能性。

总结:统一的术语和定义应该有助于治疗嗜酸性粒细胞增多症的医生。尽管取得了很大进展,但血清生物标志物与疾病严重程度相关,并可预测治疗反应,这是目前所需要的。对于 PDGFRA 阴性 HES 的理解和特定治疗也有很大的需求。

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