Eosinophilic Myeloid Disorders
Eosinophilic Myeloid Disorders
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DOI：
10.1053/j.seminhematol.2012.01.008
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发表时间：
2012-04-01
影响因子：
3.6
通讯作者：
Noel, Pierre
中科院分区：
文献类型：
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作者：
Noel, Pierre
涉及血小板源性生长因子受体α和β（PDGFRA和PDGFRB）的治疗相关突变的发现改变了我们评估和治疗克隆性嗜酸性粒细胞增多症患者的方式。尽管我们对克隆性嗜酸性粒细胞增多症的病理学有了进一步的了解，但超过 50% 的患者被诊断患有特发性疾病，10% 至 20% 患有克隆性骨髓疾病，其余患者患有淋巴细胞变异。世界卫生组织的肿瘤分类认识到嗜酸性粒细胞骨髓疾病半分子分类的重要性，并将其分为两个主要亚组：（1）伴有嗜酸性粒细胞增多和PDGFRA、PDGFRB或成纤维细胞生长因子受体1（FGFR1）异常的骨髓和淋巴肿瘤； (2) 慢性嗜酸性粒细胞白血病，除非另有说明。一个关键的挑战仍然是在克隆性嗜酸性粒细胞增多症发病机制尚不清楚的患者中鉴定酪氨酸激酶反应性分子病变。塞明赫马托尔 49：120-127。 (C) 2012 Elsevier Inc. 保留所有权利。
The discovery of therapeutically relevant mutations involving platelet-derived growth factor receptors alpha and beta (PDGFRA and PDGFRB) changed the way we evaluate and treat patients with clonal eosinophilia. Despite our improved understanding of the pathobiology of clonal eosinophilia, more than 50% of patients are diagnosed with idiopathic disease, 10% to 20% with a clonal myeloid disorder, and the remainder with a lymphocytic variant. The World Health Organization classification of tumors recognized the importance of a semi-molecular classification of eosinophilc myeloid disorders and divided them into two major subgroups: (1) myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, or fibroblast growth factor receptor 1 (FGFR1); and (2) chronic eosinophilic leukemia, not otherwise specified. A key challenge remains the identification of tyrosine kinase responsive molecular lesions in patients in whom the pathogenesis of clonal eosinophilia remains unclear. Semin Hematol 49:120-127. (C) 2012 Elsevier Inc. All rights reserved.