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病态造血在骨髓增生异常综合征和其他髓系肿瘤诊断中的应用:问题领域。

Dyserythropoiesis in the diagnosis of the myelodysplastic syndromes and other myeloid neoplasms: problem areas.

机构信息

University of Rennes, Rennes, France.

University of Rochester, New York, NY, USA.

出版信息

Br J Haematol. 2018 Aug;182(4):526-533. doi: 10.1111/bjh.15435. Epub 2018 Jun 19.

DOI:10.1111/bjh.15435
PMID:29917221
Abstract

An evaluation of the significance of specified dyserythropoietic features in suspected myelodysplastic syndrome (MDS) and acute myeloid leukaemia with erythroid dysplasia was made by means of evaluation of 100 electronic images of bone marrow erythroblasts from each of 20 subjects: 11 with a myeloid neoplasm, six with another condition that could cause erythroid dysplasia and three healthy controls. The evaluation was carried out independently by seven experienced haematologists/haematopathologists who were blinded to the diagnosis. The majority of the dyserythropoietic features listed in the World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues were validated, although karyorrhexis was found to be infrequent and lacking in specificity; multinuclearity and megaloblastosis were more often observed but also lacked specificity. Good majority agreement on the identification of dysplastic features was obtained. Despite this, it was demonstrated that a reliable diagnosis of MDS can often not be made on the basis of erythroid morphology alone. Interpretation of dyserythropoiesis must be carried out with full knowledge of other clinicopathological features and with a constant awareness of the other conditions that can be confused with MDS. An iron stain is essential, as cases with ring sideroblasts may otherwise not be recognised as having MDS.

摘要

通过对 20 名受试者(11 名患有髓系肿瘤、6 名患有其他可能导致红细胞发育不良的疾病和 3 名健康对照者)的骨髓红细胞的 100 个电子图像进行评估,评估了疑似骨髓增生异常综合征(MDS)和伴红细胞发育不良的急性髓系白血病中特定红细胞发育不良特征的意义。评估由七名经验丰富的血液学家/血液病理学家独立进行,他们对诊断结果不知情。尽管核碎裂被发现罕见且特异性不足,但大多数列入世界卫生组织造血和淋巴组织肿瘤分类的红细胞发育不良特征得到了验证;多核和巨幼红细胞增多症更常见,但也缺乏特异性。对识别发育不良特征的一致性很高。尽管如此,研究表明,仅根据红细胞形态通常无法可靠地诊断 MDS。必须充分了解其他临床病理特征,并始终意识到可能与 MDS 混淆的其他疾病,才能对红细胞发育不良进行解释。铁染色至关重要,否则可能无法识别环形铁幼粒细胞增多症病例是否患有 MDS。

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