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双表达体扩散大B细胞淋巴瘤中星空模式的临床病理学和遗传特征
Hyun-Jung Sung1, Deokhoon Kim1, Dok-Hyun Yoon2
1Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, 05505, South Korea.
Human pathology
|July 30, 2023
概括
双表达性淋巴瘤 (DEL),一种扩散性大B细胞淋巴瘤 (DLBCL) 的星空模式 (SSP),表明具有攻击性特征和较差的生存结果. 这种形态发现与遗传突变和预后不佳的临床指标相关.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 病理学 病理学 病理学
背景情况:
- 双表达性淋巴瘤 (DEL) 是扩散性大B细胞淋巴瘤 (DLBCL) 的高风险亚型.
- MYC和BCL2蛋白的同时表达定义了DEL,通常与预后不佳有关.
- 缺少用于DEL评估的标准化形态标准.
研究的目的:
- 为了研究星空模式 (SSP) 在DEL的预后意义.
- 为了将SSP与DEL病例中的临床病理和遗传特征相关联.
- 确定SSP与无进展生存率 (PFS) 和整体生存率 (OS) 的关联.
主要方法:
- 追溯分析153个DEL案件.
- 对SSP存在的形态评估.
- 与临床病理学参数 (例如c-MYC,BCL2,CD5表达,IPI得分) 的相关性分析.
- 单变量和多变量生存分析 (PFS和OS).
- 用于基因突变分析的下一代测序.
主要成果:
- SSP与攻击性特征有显著的关联:c-MYC过度表达,CD5表达和更高的IPI得分.
- 存在SSP与不利的PFS相关 (p=0.040) 和趋向于不利的OS (p=0.061).
- 在c-MYC过度表达的情况下,SSP显著预测了劣质的OS (p=0.019).
- 多变量分析证实SSP是PFS差的预测因素 (p=0.048).
- 与非SSP的DEL相比,SSP与KMT2D突变和不同的遗传特征有关.
结论:
- 星空图案可以作为双表达性淋巴瘤侵略性的形态指标.
- SSP与DEL的不良临床和遗传特征有关.
- 确定SSP可以有助于对DEL患者的风险分层和预后.
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