Falini Brunangelo, Tiacci Enrico, Martelli Maria Paola, Ascani Stefano, Pileri Stefano A
Institute of Hematology, University of Perugia, Perugia, Italy.
Discov Med. 2010 Oct;10(53):281-92.
The World Health Organization (WHO) classification of lympho-hematopoietic neoplasms is increasingly based on genetic criteria. Here, we focus on changes that, as compared to the 2001 edition, were introduced into the 2008 WHO classification of acute myeloid leukemia (AML) and related precursor neoplasms. The category of AML with recurrent genetic abnormalities was expanded to account for 60% of AML by adding three distinct entities, i.e., AML with t(6,9), inv(3), or t(1;22), and two provisional entities, i.e., AML with mutated NPM1 or CEBPA. These changes have greatly modified the approaches to diagnosis and prognostic stratification of AML patients. To emphasize the need of various parameters for diagnosis, including myelodysplasia (MD)-related cytogenetic abnormalities, history of myelodysplasia or myelodysplasia/myeloproliferative neoplasm, and multilineage dysplasia, the category of "AML with multilineage dysplasia" was re-named AML with MD-related changes. Finally, we describe the unique characteristics of myeloid proliferations associated with Down syndrome and blastic plasmacytoid dendritic cell neoplasm.
世界卫生组织(WHO)对淋巴造血系统肿瘤的分类越来越多地基于遗传学标准。在此,我们重点关注与2001年版相比,被纳入2008年WHO急性髓系白血病(AML)及相关前驱肿瘤分类中的变化。伴有复发性遗传学异常的AML类别通过增加三个不同实体,即伴有t(6;9)、inv(3)或t(1;22)的AML,以及两个暂定实体,即伴有NPM1或CEBPA突变的AML,扩展至占AML的60%。这些变化极大地改变了AML患者的诊断方法和预后分层。为强调诊断所需的各种参数,包括与骨髓发育异常(MD)相关的细胞遗传学异常、骨髓发育异常或骨髓发育异常/骨髓增殖性肿瘤病史以及多系发育异常,“伴有多系发育异常的AML”类别被重新命名为伴有MD相关改变的AML。最后,我们描述了与唐氏综合征相关的髓系增殖以及母细胞性浆细胞样树突状细胞瘤的独特特征。