PROPOSALS FOR THE CLASSIFICATION OF THE MYELODYSPLASTIC SYNDROMES
PROPOSALS FOR THE CLASSIFICATION OF THE MYELODYSPLASTIC SYNDROMES
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DOI：
10.1111/j.1365-2141.1982.tb08475.x
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发表时间：
1982-01-01
影响因子：
6.5
通讯作者：
SULTAN, C
中科院分区：
文献类型：
--
作者：
BENNETT, JM;CATOVSKY, D;SULTAN, C
概述：提出了诊断各种骨髓增生异常综合征（MDS）的新的诊断标准，并详细描述了可能有助于定义MDS的特征。描述了五种MDS：（1）难治性贫血（RA），（2）RA伴环形铁粒幼细胞，（3）RA伴原始细胞过多（RAEB），（4）慢性粒单核细胞白血病（CMML），和（5）RAEB“转化中”。这些病症的主要区别特征之一是外周血（PB）和/或骨髓（BM）中母细胞的比例。具有诊断重要性的胚细胞的形态学特征已被重新定义。在RA中，有或没有环形铁粒幼细胞，PB中的原始细胞少于1%，BM中的原始细胞少于5%; RAEB定义为BM中的原始细胞介于5%和20%之间，PB中的原始细胞少于5%; RAEB在转化中（新定义的类别）将在存在以下任何一个特征时予以考虑：（i）PB中原始细胞超过5%，（ii）BM中20 - 30%，（iii）BM或PB中粒细胞前体中存在奥尔杆。根据这些新定义的标准，现在建议超过30%的骨髓原始细胞足以诊断任何形式的急性髓性白血病（AML）（M1-M6）。MDS的拟议描述应有助于解释细胞遗传学和骨髓培养研究中出现的数据，并寻找可能具有预后意义的特征。认识到新的类别，RAEB的转化，可能有助于阐明AML的发病机制。
Summary.New, diagnostic criteria for the diagnosis of the various myelodysplastic syndromes (MDS) are proposed, and a detailed description is given of the features that may help define MDS. Five MDS are described: (1) refractory anaemia (RA), (2) RA with ring sideroblasts, (3) RA with excess of blasts (RAEB), (4) chronic myelomonocytic leukaemia (CMML), and (5) RAEB ‘in transformation’. One of the main distinguishing features of these conditions is the proportion of blast cells in the peripheral blood (PB) and/or bone marrow (BM). The morphological features of the blast cells that are of diagnostic importance have been redefined. In RA, with or without ringed sideroblasts, there are fewer than 1% of blasts in the PB and fewer than 5% in the BM; RAEB is defined as having between 5% and 20% of blasts in the BM and fewer than 5% in the PB; RAEB in transformation (a newly defined category) will be considered when any one of the following features is present: (i) more than 5% of blasts in the PB, (ii) 20‐30% in the BM, and (iii) the presence of Auer rods in granulocyte precursors in BM or PB. In accordance with these newly defined criteria, it is now proposed that over 30% of bone marrow blasts will suffice for the diagnosis of acute myeloid leukaemia (AML) in any of its forms (M1‐M6). The proposed descriptions of the MDS should facilitate the interpretation of data emerging from cytogenetic and bone marrow culture studies and the search for features of possible prognostic significance. Recognition of the new category, RAEB in transformation, may throw light on the pathogenesis of AML.