Havelange Violaine, Demoulin Jean-Baptiste
de Duve Institute, Université catholique de Louvain, Brussels, Belgium ; Department of Hematology, Cliniques universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium.
J Blood Med. 2013 Aug 9;4:111-21. doi: 10.2147/JBM.S33142. eCollection 2013.
Recent advances in our understanding of the molecular mechanisms underlying hypereosinophilia have led to the development of a 'molecular' classification of myeloproliferative disorders with eosinophilia. The revised 2008 World Health Organization classification of myeloid neoplasms included a new category called "myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1." Despite the molecular heterogeneity of PDGFR (platelet-derived growth factor receptor) rearrangements, tyrosine kinase inhibitors at low dose induce rapid and complete hematological remission in the majority of these patients. Other kinase inhibitors are promising. Further discoveries of new molecular alterations will direct the development of new specific inhibitors. In this review, an update of the classifications of myeloproliferative disorders associated with hypereosinophilia is discussed together with open and controversial questions. Molecular mechanisms and promising results of tyrosine kinase inhibitor treatments are reviewed.
近年来,我们对嗜酸性粒细胞增多症潜在分子机制的理解取得了进展,这促使了对伴有嗜酸性粒细胞增多的骨髓增殖性疾病进行“分子”分类。2008年世界卫生组织修订的髓系肿瘤分类中新增了一个类别,称为“伴有嗜酸性粒细胞增多以及PDGFRA、PDGFRB或FGFR1异常的髓系和淋系肿瘤”。尽管血小板衍生生长因子受体(PDGFR)重排存在分子异质性,但低剂量的酪氨酸激酶抑制剂可使大多数此类患者迅速实现完全血液学缓解。其他激酶抑制剂也颇具前景。新分子改变的进一步发现将指导新型特异性抑制剂的研发。在本综述中,我们将讨论与嗜酸性粒细胞增多相关的骨髓增殖性疾病分类的更新情况,以及一些尚未解决的争议性问题。同时,还将对酪氨酸激酶抑制剂治疗的分子机制和有前景的结果进行综述。