CD371阳性儿科B细胞急性淋巴细胞白血病:倾向于谱系切换和缓慢的治疗早期反应
Barbara Buldini1,2, Elena Varotto1, Margarita Maurer-Granofszky3
1Pediatric Hematology, Oncology and Stem Cell Transplant Division, Maternal and Child Health Department, University of Padua, Padua, Italy.
Blood
|January 12, 2024
概括
骨髓单细胞标记物CD371识别了儿科B细胞前体急性淋巴细胞白血病 (BCP-ALL) 的一个子集,具有过渡性骨髓单细胞切换和劣质早期反应. 尽管如此,CD371阳性BCP-ALL在最小的残留疾病导向疗法下显示出良好的结果.
科学领域:
- 血液学 血液学 血液学
- 儿科瘤学 儿科瘤学
- 免疫型定型 免疫型定型
背景情况:
- 准确的免疫类型和最小残留疾病 (MRD) 评估对于急性淋巴细胞白血病 (ALL) 治疗至关重要.
- 国际柏林-法兰克福-斯特 (iBFM) 流量网络引入了骨髓单细胞标记物CD371以改善ALL的表征.
研究的目的:
- 研究CD371阳性 (CD371pos) 儿科B细胞前体ALL (BCP-ALL) 的临床和生物特征.
- 评估CD371表达与儿科BCP-ALL治疗反应和存活率相关的意义.
主要方法:
- 来自AIEOP-BFM ALL 2009试验的1812名儿科BCP-ALL患者的评估 (查和验证队列).
- 实验室评估包括诊断时的形态,免疫类型和遗传分析.
- 响应评估使用了形态学,多参数流细胞计 (MFC) 和聚合酶链反应 (PCR) -MRD.
主要成果:
- CD371pos BCP-ALL (8.8%) 与年龄较大,ETV6::RUNX1频率较低,免疫类型不成熟以及CD34/CD45表达相关.
- CD371pos BCP-ALL表现出暂时的骨髓单细胞切换 (高达65.4%) 和较差的早期化疗反应.
- 五年无事件生存率为88.3%;96.4%的DUX4融合阳性病例是CD371pos.
结论:
- CD371是儿科BCP-ALL中过渡性骨髓单细胞切换最敏感的标志物,对于准确的MFCMRD评估至关重要.
- CD371pos与早期治疗反应不佳有关,但使用基于MRD的疗法可以获得良好的结果.
- CD371有助于识别特定的BCP-ALL亚组,需要量身定制的治疗策略.
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