Stanford University School of Medicine, Stanford Cancer Center, Stanford, California, USA.
Curr Opin Hematol. 2010 Mar;17(2):117-24. doi: 10.1097/MOH.0b013e3283366c70.
The aim of this brief review is to evaluate recent developments in the classification and treatment of eosinophilic myeloid disorders in the context of reactive, lymphocyte-variant, and idiopathic eosinophilias.
The revised 2008 WHO classification recognizes both molecularly defined ('myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1') and undefined (chronic eosinophilic leukemia, not otherwise specified) eosinophilic myeloid disorders. An increasingly sophisticated understanding of the molecular underpinnings of eosinophilia has translated into rational use of biologically targeted therapies such as imatinib mesylate. Conventional cytotoxics and interferon-alpha still have an established role in treating these diseases. Although studied in idiopathic hypereosinophilic syndrome, the therapeutic niche of anti-interleukin-5 (mepolizumab) and anti-CD52 (alemtuzumab) antibody therapy in eosinophilic myeloid diseases has yet to be established.
Molecular/genetic analysis is now mandatory for the diagnosis, classification, and treatment of eosinophilic myeloid disorders. The finding of rearranged, constitutively activated PDGFRA/B identifies patients who are eminently treatable with tyrosine kinase inhibitors.
本综述旨在评估在反应性、淋巴细胞变异型和特发性嗜酸性粒细胞增多症的背景下，嗜酸性粒细胞髓系疾病分类和治疗的最新进展。
修订后的 2008 年世界卫生组织分类既承认分子定义的（伴有嗜酸粒细胞增多和 PDGFRA、PDGFRB 或 FGFR1 异常的髓系和淋巴系肿瘤）和未定义的（慢性嗜酸粒细胞白血病，未另作说明）嗜酸性粒细胞髓系疾病。对嗜酸粒细胞增多症的分子基础的理解日益深入，已转化为对生物靶向治疗的合理应用，如伊马替尼甲磺酸盐。传统细胞毒素和干扰素-α在治疗这些疾病方面仍然具有既定的作用。虽然在特发性嗜酸性粒细胞增多综合征中进行了研究，但抗白细胞介素-5（美泊利单抗）和抗 CD52（阿仑单抗）抗体治疗在嗜酸性粒细胞髓系疾病中的治疗作用尚未确定。
目前，对于嗜酸性粒细胞髓系疾病的诊断、分类和治疗，分子/遗传分析是强制性的。发现重排的、组成性激活的 PDGFRA/B 可识别出用酪氨酸激酶抑制剂治疗效果显著的患者。
