Hypereosinophilic syndrome: An update
Hypereosinophilic syndrome: An update
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嗜酸性粒细胞增多综合征：更新
DOI：
10.1002/ajh.20423
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发表时间：
2005
影响因子：
12.8
通讯作者：
W. Williams
中科院分区：
文献类型：
--
作者：
H. J. Wilkins;M. Crane;K. Copeland;W. Williams
嗜酸性粒细胞增多综合征（HES）是一种罕见的疾病，其特征是持续和显著的嗜酸性粒细胞增多合并器官系统功能障碍。HES具有很大的临床异质性，但如果不进行治疗，可能会致命，特别是对于患有该疾病骨髓增生异常变异的患者。虽然HES的病理生理学定义不明确，但导致嗜酸性粒细胞成熟的细胞因子（白细胞介素5 [IL-5]、IL-3、粒细胞-巨噬细胞集落刺激因子[GM-CSF]）失调是主要特征。在这些细胞因子中，IL-5似乎在调节嗜酸性粒细胞成熟中发挥最大作用。目前尚无美国食品药品监督管理局（FDA）批准的HES治疗方法;目前的策略旨在降低血液嗜酸性粒细胞，并试图限制终末器官损伤。从历史上看，皮质类固醇和细胞毒性药物一直是治疗的主要药物，生物反应调节剂如干扰素α在某些患者中也有效。然而，尽管生存率有所改善，但现有药物在疗效、耐受性和长期毒性方面存在显著局限性。最近，已经开发了针对HES发病机制中特定靶点的新药物。这些包括甲磺酸伊马替尼，一种酪氨酸激酶抑制剂，以及最近的mepolizumab，一种抗IL-5单克隆抗体。在一个小的患者病例系列中，这些药物已被证明在HES患者中产生血液学和临床应答，尽管它们在不同的患者亚组中可能有效。这些靶向治疗有可能改善临床结局，并进一步了解这种难以治疗的疾病的病理生理学。Am. J. Hematol. 80：148-157，2005.© 2005 Wiley利斯公司
Hypereosinophilic syndrome (HES) is a rare disorder that is characterized by persistent and marked eosinophilia combined with organ system dysfunction. HES has substantial clinical heterogeneity but can be fatal without treatment, especially in patients who present with a myelodysplastic variant of the disorder. Although the pathophysiology of HES is poorly defined, dysregulation of cytokines (interleukin 5 [IL‐5], IL‐3, granulocyte‐macrophage colony‐stimulating factor [GM‐CSF]) responsible for the maturation of eosinophils is a primary feature. Of these cytokines, IL‐5 appears to have the greatest role in the regulation of eosinophil maturation. There is no Food and Drug Administration‐approved treatment for HES as yet; current strategies are designed to lower blood eosinophils and attempt to limit end‐organ damage. Historically, corticosteroids and cytotoxic agents have been the mainstays of therapy, with biological response modifiers such as interferon‐alpha also effective in some patients. However, despite improvements in survival, available agents have significant limitations in terms of efficacy, tolerability, and long‐term toxicity. More recently, new agents directed at specific targets in the pathogenesis of HES have been developed. These include imatinib mesylate, a tyrosine kinase inhibitor, and more recently, mepolizumab, an anti‐IL‐5 monoclonal antibody. In a small case series of patients, these agents have been shown to produce hematological and clinical responses in patients with HES, although they may be effective in different subsets of patients. These targeted therapies have the potential to improve clinical outcomes and to further the understanding the pathophysiology of this difficult‐to‐treat condition. Am. J. Hematol. 80:148–157, 2005. © 2005 Wiley‐Liss, Inc.
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作者：
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