Kuriyama Kazutaka
Hematoimmunology, School of Health Science, Faculty of Medicine, University of the Ryukyus.
Nihon Rinsho. 2009 Oct;67(10):1853-62.
Myeloid leukemia in this series corresponds to the myeloid neoplasms of the 4th WHO classification of pathology and genetics of tumor of haematopoietic and lymphoid tissue. The myeloid neoplasms are composed of six categories, which are 1) myeloproliferative neoplasms (MPN), a new category of 2) myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1, 3) myelodysplastic syndrome (MDS)/MPN, 4) MDS, 5) acute myeloid leukemia (AML) and related precursor neoplasms, and 6) acute leukemias of ambiguous lineage. In MPNs without chronic myelogenous leukemia, the genetic marker of JAK2 V617F is added to the diagnostic criteria for polycythemia vera, essential thrombocythemia and primary myelofibrosis. MDS has the new subtype of refractory cytopenia with unilineage dysplasia composed of refractory anemia, refractory neutropenia and refractory thrombocytopenia. AML with t(9; 11) (p22;q23); MLLT3-MLL, AML with t(6;9) (p23; q34); DEK-NUP214, AML with inv(3) (q21q26.2) or t(3; 3) (q21 ; q26.2); RPN1-EVI1 and AML (megakaryoblastic) with t(1; 22) (p13; q13); RBM15-MKL1 are added to the subtype of AML with recurrent genetic abnormalities, and AML with gene mutations of NPM1 and CEBPA are also added as provisional entities of it. The myeloid neoplasms of the 4th WHO classification are comprehensive and seem to be dynamic by incorporating the results of leukemia researches.
本系列中的髓系白血病对应于世界卫生组织(WHO)造血与淋巴组织肿瘤病理学和遗传学第4版分类中的髓系肿瘤。髓系肿瘤由六类组成,分别为:1)髓系增殖性肿瘤(MPN);2)伴有嗜酸性粒细胞增多以及血小板衍生生长因子受体α(PDGFRA)、血小板衍生生长因子受体β(PDGFRB)或成纤维细胞生长因子受体1(FGFR1)异常的髓系和淋巴系肿瘤,这是一个新类别;3)骨髓增生异常综合征/髓系增殖性肿瘤(MDS/MPN);4)骨髓增生异常综合征(MDS);5)急性髓系白血病(AML)及相关前驱肿瘤;6)谱系不明的急性白血病。在不伴有慢性粒细胞白血病的MPN中,JAK2 V617F基因标记被纳入真性红细胞增多症、原发性血小板增多症和原发性骨髓纤维化的诊断标准。MDS有新的难治性单系发育异常性血细胞减少亚型,由难治性贫血、难治性中性粒细胞减少和难治性血小板减少组成。伴有t(9;11)(p22;q23);MLLT3-MLL的AML、伴有t(6;9)(p23;q34);DEK-NUP214的AML、伴有inv(3)(q21q26.2)或t(3;3)(q21;q26.2);RPN1-EVI1的AML以及伴有t(1;22)(p13;q13);RBM15-MKL1的急性巨核细胞白血病被添加到伴有复发性基因异常的AML亚型中,伴有核仁磷酸蛋白1(NPM1)和CCAAT增强子结合蛋白α(CEBPA)基因突变的AML也被添加为其暂定实体。WHO第4版分类中的髓系肿瘤具有综合性,并且通过纳入白血病研究结果似乎具有动态性。