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2008 年 WHO 分类标准下骨髓增生异常综合征(MDS)诊断的观察者间差异。

Inter-observer variance with the diagnosis of myelodysplastic syndromes (MDS) following the 2008 WHO classification.

机构信息

Department of Hematology, Hospital General Universitario Gregorio Marañon, Madrid, Spain.

出版信息

Ann Hematol. 2013 Jan;92(1):19-24. doi: 10.1007/s00277-012-1565-4. Epub 2012 Sep 5.

Abstract

Morphology is the basis of the diagnosis of myelodysplastic syndromes (MDS). The WHO classification offers prognostic information and helps with the treatment decisions. However, morphological changes are subject to potential inter-observer variance. The aim of our study was to explore the reliability of the 2008 WHO classification of MDS, reviewing 100 samples previously diagnosed with MDS using the 2001 WHO criteria. Specimens were collected from 10 hospitals and were evaluated by 10 morphologists, working in five pairs. Each observer evaluated 20 samples, and each sample was analyzed independently by two morphologists. The second observer was blinded to the clinical and laboratory data, except for the peripheral blood (PB) counts. Nineteen cases were considered as unclassified MDS (MDS-U) by the 2001 WHO classification, but only three remained as MDS-U by the 2008 WHO proposal. Discordance was observed in 26 of the 95 samples considered suitable (27 %). Although there were a high number of observers taking part, the rate of discordance was quite similar among the five pairs. The inter-observer concordance was very good regarding refractory anemia with excess blasts type 1 (RAEB-1) (10 of 12 cases, 84 %), RAEB-2 (nine of 10 cases, 90 %), and also good regarding refractory cytopenia with multilineage dysplasia (37 of 50 cases, 74 %). However, the categories with unilineage dysplasia were not reproducible in most of the cases. The rate of concordance with refractory cytopenia with unilineage dysplasia was 40 % (two of five cases) and 25 % with RA with ring sideroblasts (two of eight). Our results show that the 2008 WHO classification gives a more accurate stratification of MDS but also illustrates the difficulty in diagnosing MDS with unilineage dysplasia.

摘要

形态学是骨髓增生异常综合征(MDS)诊断的基础。世界卫生组织(WHO)分类提供预后信息,并有助于治疗决策。然而,形态学变化可能存在潜在的观察者间差异。我们的研究旨在探讨 2008 年 WHO MDS 分类的可靠性,通过对使用 2001 年 WHO 标准诊断为 MDS 的 100 例样本进行回顾性分析。标本取自 10 家医院,由 10 名形态学家进行评估,这些形态学家分为 5 对。每位观察者评估 20 例样本,每位形态学家对 20 例样本中的 10 例进行独立分析。第二位观察者对临床和实验室数据除外周血(PB)计数外一概不知情。根据 2001 年 WHO 分类,有 19 例被认为是未分类 MDS(MDS-U),但根据 2008 年 WHO 建议,只有 3 例仍被归类为 MDS-U。在 95 例被认为合适的样本中,有 26 例出现了不一致(27%)。尽管参与的观察者人数众多,但五对之间的不一致率非常相似。对于难治性贫血伴原始细胞过多 1 型(RAEB-1)(12 例中的 10 例,84%)、RAEB-2(10 例中的 9 例,90%),观察者之间的一致性非常好,对于难治性血细胞减少伴多系发育异常(50 例中的 37 例,74%),一致性也很好。然而,在大多数情况下,具有单系发育异常的分类无法重现。难治性血细胞减少伴单系发育异常的一致性率为 40%(5 例中的 2 例),难治性贫血伴环形铁幼粒细胞的一致性率为 25%(8 例中的 2 例)。我们的结果表明,2008 年 WHO 分类对 MDS 进行了更准确的分层,但也说明了诊断具有单系发育异常的 MDS 的困难。

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