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Acute Erythroleukemias, Acute Megakaryoblastic Leukemias, and Reactive Mimics: A Guide to a Number of Perplexing Entities.

急性红白血病、急性巨核细胞白血病及其反应性类似病变等复杂病变诊断指南

Wang SA,Hasserjian RP

Abstract

At the 2013 Society for Hematopathology/European Association for Hematopathology Workshop, 36 cases were submitted to the session that covered acute erythroid leukemia (AEL), acute megakaryoblastic leukemia (AMKL), and reactive mimics.
Cases were reviewed by the session chairs and workshop panel to reach a consensus diagnosis.
For acute erythroleukemia, erythroid/myeloid type, discussion acknowledged overlapping features between AEL and myelodysplastic syndromes. Cases submitted as pure erythroid leukemia had distinctive morphology and immunophenotype, complex karyotypes, and aggressive clinical behavior, illustrating certain diagnostic features not currently captured by the current World Health Organization (WHO) definition. In Down syndrome, there were striking similarities between transient abnormal myelopoiesis and AMKL. Most cases of AMKL in adults would be classified as acute myeloid leukemia with myelodysplasia-related changes according to the WHO classification, but this approach deemphasizes their unique clinical, morphologic, and immunophenotypic features.
The broad spectrum of cases illustrated the difficulties and complex issues involved in establishing a diagnosis of these entities and the need for better disease definitions.

摘要

2013年血液病理学会/欧洲血液病理协会会议上提交并讨论了36个病例,涵盖急性红白血病(AEL)、急性巨核细胞白血病(AMKL)及其反应性类似病变。

大会主席团和工作小组复查了36个病例,对病变的诊断达成共识。

对于红系型/髓系型急性红白血病,大会承认AEL和骨髓增生异常综合症两者特征有重叠。大会提交的纯红白血病病例有独特的形态学改变、免疫表型、染色体复杂核型和侵袭性临床行为,某些诊断特征目前尚未被WHO收录。对于唐氏综合症患者,一过性骨髓细胞生成异常和AMKL有惊人的相似之处。根据WHO分类标准,大多数成人AMKL病例被归类为急性髓系白血病伴骨髓增生异常相关性改变,但这种归类忽略了病变独特的临床、形态学及其免疫表型特征。

上述病变谱系宽泛,解释了病变诊断标准建立的困难和复杂性,需要更合理地定义疾

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