真性红细胞增多症后骨髓纤维化引起的复杂性嗜酸性粒细胞增多症：一例对伊马替尼有反应的病例。
Complex hypereosinophilia arising from post-polycythemia vera myelofibrosis: A case of imatinib-responsiveness.
作者信息
Gentzler Ryan D, Minella Alex C, Stein Brady L
机构信息
Department of Medicine, Division of Hematology/Oncology, Robert H. Lurie Comprehensive Cancer Center, Northwestern University Feinberg School of Medicine, USA.
出版信息
Leuk Res Rep. 2012 Nov 10;1(1):9-12. doi: 10.1016/j.lrr.2012.07.001. eCollection 2012.
The classical myeloproliferative neoplasms (MPNs) feature an overproduction of mature blood elements. Phenotypic conversion, including transformation to myelofibrosis (MF) in those with antecedent ET and PV is a feared complication. Hypereosinophilic syndromes (HESs), especially those with myeloproliferative variants, can display similar features, including organomegaly, marrow fibrosis, clonality, thrombotic tendencies, and acute myeloid leukemia (AML) transformation. However, this group of illnesses is typically clinically and molecularly distinct from the classical MPNs. We report a case of a 59-yr-old woman with complex hypereosinophilia in the setting of post-polycythemic myelofibrosis (post-PVMF), with multi-system end-organ damage characteristic of HES.
经典型骨髓增殖性肿瘤（MPNs）的特征是成熟血细胞过度生成。表型转化，包括先前患有真性红细胞增多症（ET）和原发性血小板增多症（PV）的患者转化为骨髓纤维化（MF），是一种令人担忧的并发症。高嗜酸性粒细胞综合征（HESs），尤其是那些具有骨髓增殖性变异型的，可表现出类似特征，包括器官肿大、骨髓纤维化、克隆性、血栓形成倾向以及急性髓系白血病（AML）转化。然而，这组疾病在临床和分子水平上通常与经典型MPNs不同。我们报告一例59岁女性，在真性红细胞增多症后骨髓纤维化（post-PVMF）背景下出现复杂性高嗜酸性粒细胞增多症，具有HES的多系统终末器官损害特征。
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