在年轻成人中,红系显性髓系肿瘤和过多的母细胞表现出不同的遗传谱。

IF 2.3 3区 医学 Q2 HEMATOLOGY
Laurène Fenwarth, Benjamin Podvin, Loïc Vasseur, Delphine Lebon, Guillaume Couillez, Céline Berthon, Alexis Caulier, Elise Fournier, Claire Bories, Benjamin Carpentier, Sabine Tricot, Kevin-James Wattebled, Catherine Roche-Lestienne, Valentin Lestringant, Carine Hauspie, Karine Celli-Lebras, Maël Heiblig, Hervé Dombret, Raphael Itzykson, Jean-Pierre Marolleau, Loïc Garçon, Claude Preudhomme, Nicolas Duployez, Thomas Boyer
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引用次数: 0

摘要

急性髓性白血病(AML)分类的演变已逐渐将诊断重点转向遗传标准。尽管如此,形态学仍然是临床实践中的一个关键因素,通常作为附加分子研究的初始触发因素。急性红细胞白血病(AEML)的诊断,最初由FAB组定义,在最新的WHO和ICC分类中不再被认为是一个独特的实体。一些研究表明,AEML与骨髓增生异常肿瘤有相似之处,包括高频率的TP53突变和不良核型。在这里,我们对成人AEML进行了回顾性分析,该AEML采用历史形态学标准(红细胞前体≥50%,非红细胞母细胞≥20%)定义。与老年患者相比,年轻人(18-60岁)表现出独特的遗传谱,包括正常核型(65%)、NPM1(35%)和UBTF(23%)突变的高患病率。npm1突变病例的AEML形态不影响临床结果,但与特定的分子特征相关,包括WT1的富集和内聚蛋白基因突变。在这个年龄组中,我们的研究结果支持,根据当前的遗传标准,形态学上定义的AEML通常对应于AML,这与最近优先考虑分子特征而不是形态学的分类系统一致。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Myeloid Neoplasms With Erythroid Predominance and Excess Blasts in Young Adults Exhibit Distinct Genetic Profiles.

The evolution of acute myeloid leukemia (AML) classifications has progressively shifted the diagnostic focus toward genetic criteria. Nevertheless, morphology remains a key element in clinical practice, often serving as the initial trigger for additional molecular investigations. The diagnosis of acute erythroleukemia (AEML), initially defined by the FAB group, is no longer recognized as a distinct entity in the latest WHO and ICC classifications. Some studies have indicated that AEML shares similarities with myelodysplastic neoplasms, including a high frequency of TP53 mutations and adverse karyotypes. Here, we conducted a retrospective analysis in adults with AEML defined using historical morphologic criteria (≥ 50% erythroid precursors and ≥ 20% blasts among non-erythroid cells). In contrast to older patients, young adults (18-60 years) exhibit unique genetic profiles including a high prevalence of normal karyotypes (65%), NPM1 (35%) and UBTF (23%) mutations. AEML morphology in NPM1-mutated cases did not impact clinical outcomes but was associated with specific molecular features, including an enrichment of WT1 and cohesin gene mutations. In this age group, our findings support that morphologically defined AEML often corresponds to AML according to current genetic criteria, consistent with recent classification systems that prioritize molecular features over morphology.

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来源期刊
CiteScore
5.50
自引率
0.00%
发文量
168
审稿时长
4-8 weeks
期刊介绍: European Journal of Haematology is an international journal for communication of basic and clinical research in haematology. The journal welcomes manuscripts on molecular, cellular and clinical research on diseases of the blood, vascular and lymphatic tissue, and on basic molecular and cellular research related to normal development and function of the blood, vascular and lymphatic tissue. The journal also welcomes reviews on clinical haematology and basic research, case reports, and clinical pictures.
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