骨髓状新生体与寡聚单细胞症呈现异质的病理和遗传特征
Vandana Baloda1, Raniah Al Amri2, Pranav P Patwardhan3
1Department of Pathology, UPMC, Pittsburgh, Pennsylvania; Now with Memorial Sloan Kettering Cancer Center, New York, New York.
概括
髓瘤新分类对慢性骨髓单细胞白血病 (CMML) 的诊断产生影响. 虽然WHO5增加了CMML病例,但ICC标准改进了诊断,强调了基因而不是形态学,以便更好地预测结果.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 遗传学 是一个遗传学.
背景情况:
- 骨髓瘤瘤的分类标准,包括慢性骨髓单细胞白血病 (CMML),已在世界卫生组织分类 (WHO5) 和国际共识分类 (ICC) 的第五版中更新.
- 世卫组织5和ICC都将绝对单细胞数值值降至0.5 × 109/L用于CMML诊断.
- 国际医学会为CMML引入了新的形态学标准,与世卫组织5.5的标准不同.
研究的目的:
- 评估WHO5和ICC分类变化的影响,对以前在WHO4r分类的骨髓瘤的一组产生影响.
- 评估修订后的单细胞计数和新的形态学标准如何影响骨髓质疏松症候群 (MDS) 重新归类为骨髓质疏松型慢性肌痛性肺炎 (MD-CMML).
主要方法:
- 对大量骨髓瘤瘤队列的分析 (WBC < 13 × 10 < 9 < / L,爆发 < 20%),最初使用WHO4r进行分类.
- 根据WHO5和ICC标准对病例数量和重新分类率的比较,重点是MD-CMML.
- 对遗传特征的评估及其与与分类标准相关的结果的相关性.
主要成果:
- 较低的单细胞值可能会将大约20%的WHO4r定义的MDS重新归类为MD-CMML.
- 与WHO4r相比,WHO5标准导致MD-CMML病例增加了84%.
- 由于形态变化的原因,ICC标准显著减少了MD-CMML病例 (WHO5的107例减少到ICC的40例),并使55%的WHO4r定义的MD-CMML无法分类.
- 在ICC定义的CMML中,CMML类遗传特征的丰富性呈现出.
- 基因分类和风险模型在预测患者结果方面被证明优于单细胞计数或形态学.
结论:
- 修订的骨髓瘤瘤分类标准,特别是CMML,对诊断率产生了重大影响.
- 虽然减少了MD-CMML诊断,但ICC标准更好地与遗传特征保持一致.
- 基因组信息为CMML风险分层和结果预测提供了比传统的形态学或单细胞计数标准更强大的方法.
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