将2022年更新的世卫组织和ICC分类中的恶性单细胞症进行比较
Francis Baumgartner1,2,3, Constance Baer1, Stefanos Bamopoulos2,3
1Munich Leukemia Laboratory, Munich, Germany.
Blood
|December 8, 2023
概括
慢性骨髓单细胞白血病 (CMML) 的新分类重新定义了病例,在新分类的患者中确定了不同的遗传特征. 尽管存在差异,但新的和已建立的骨髓质疏松性-CMML组之间的生存结果仍然相似.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 分子生物学分子生物学
背景情况:
- 2022年世界卫生组织 (WHO) 和国际共识分类 (ICC) 更新了慢性骨髓单细胞白血病 (CMML) 的定义.
- 以前的分类可能已经排除了现在根据新标准认可的某些情况.
研究的目的:
- 评估2022年世卫组织和ICC分类对CMML诊断和患者护理的影响.
- 描述新分类的CMML病例的遗传和转录情景.
主要方法:
- 分析了3311个已确定的CMML病例 (世卫组织2017年) 和2130个符合新标准的寡单细胞病例.
- 适用世卫组织2022年和ICC 2022年分类系统.
- 综合性遗传,分子,免疫类型和转录组分析.
主要成果:
- 世卫组织2022年分类确定了CMML病例的1.5倍,比ICC 2022年从寡头单细胞瘤队列.
- 新归类的骨髓质疏松性-CMML病例显示出独特的突变特征,其中包括骨髓质疏松性综合征 (MDS) 典型的变化.
- 转录基因分析揭示了新分类的CMML的一个独特子组,与已确定的MD-CMML和骨髓增殖性CMML分开.
- 在新分类的和已确定的骨髓质疏松性CMML病例之间,没有观察到总生存时间的显著差异.
结论:
- 2022年的分类显著改变了CMML病例定义,特别是对于患有寡单细胞瘤的患者.
- 新发现的CMML病例代表了MDS和已确定的CMML之间的疾病连续性,具有独特的分子特征,但生存率相似.
- 这些发现强调了更新分类对于准确诊断和了解CMML异质性的重要性.
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