在呈现时,是否真的存在慢性髓性白血病的加速阶段?
Sen Yang1, Xiaoshuai Zhang1, Robert Peter Gale2
1Peking University People's Hospital, Peking University Institute of Hematology, National Clinical Research Center for Hematologic Disease, Beijing, China.
Leukemia
|December 5, 2024
概括
慢性髓性白血病 (CML) 的加速阶段可能与氨酸激酶抑制剂 (TKI) 治疗不同. 目前的数据表明,CML应该分为两个阶段:慢性阶段和急性/爆发性阶段.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 白血病研究研究 白血病研究
背景情况:
- 在诊断时,慢性髓性白血病 (CML) 中存在明显的加速阶段 (AP),特别是在氨酸激酶抑制剂 (TKI) 治疗下,仍在争论中.
- 目前的分类,如欧洲白血病网 (ELN) 和国际共识分类 (ICC),根据特定的血液学和细胞遗传学标准来定义AP.
- 了解AP的预后意义对于优化CML治疗策略和患者结果至关重要.
研究的目的:
- 研究慢性髓性白血病 (CML) 诊断时加速期 (AP) 的特异性和预后影响,特别是在氨酸激酶抑制剂 (TKI) 治疗时代.
- 评估目前的欧洲白血病网 (ELN) 和国际共识分类 (ICC) 对AP的标准.
- 建议对诊断时的CML阶段进行修订的分类系统.
主要方法:
- 追溯分析2122连续CML患者的慢性阶段 (CP) 或AP诊断,使用2020年ELN标准进行分类.
- 在CP和AP分组之间,以及在不同风险层 (ELTS) 中,比较无变异生存率 (TFS) 和总生存率 (OS).
- 评估遵守2020年ELNTKI响应里程碑及其与生存结果的相关性.
主要成果:
- 仅基于基细胞增加而被归类为AP的CML患者表现出与中等风险CP患者相似的TFS和生存率.
- 与高风险的CP患者相比,AP患者的Blaster增加有更糟糕的TFS,但相似的存活率.
- 与高风险CP患者相比,血小板减少的AP患者具有类似的TFS,但生存率较差.
- 达到ELN TKI反应里程碑的CP和AP患者的比例相似,但在未能达到这些里程碑的AP患者中观察到更糟糕的TFS和存活率.
- 使用2022年ICC标准获得了类似的发现,用额外的细胞遗传异常取代减少的血小板.
结论:
- 这些数据支持消除加速阶段 (AP) 作为CML的独特诊断类别,与2022年世卫组织分类保持一致.
- 建议修订CML分类,将疾病在诊断时分为两个阶段:慢性阶段 (CP) 和急性或爆发性阶段.
- 该研究建议将"非常高风险"队列纳入ELTS得分,包括患有血球爆炸增加或血小板减少的患者.
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