Abstract：
OBJECTIVE: To evaluate the outcomes of patients with 3q26.2/MECOM-rearranged chronic myeloid leukemia (CML).
METHODS: We reviewed consecutive adult patients with 3q26.2/MECOM-rearranged CML between January 1, 1998 and February 16, 2023. Rearrangements of 3q26.2/MECOM were confirmed by conventional cytogenetics, and fluorescence in situ hybridization starting in 2015.
RESULTS: We identified 55 patients with MECOM-rearranged CML, including 23 in chronic phase (CP) or accelerated phase (AP) and 32 in blast phase (BP). Nine patients (16%) achieved a major cytogenetic response (MCyR) or deeper. At a median follow-up of 89 months, median survival was 14 months. The 5-year survival rate was 19% overall, 23% in CML-CP/AP, and 15% in CML-BP. In the 6-month landmark analysis, the 5-year survival rate was 41% for allogeneic stem cell transplantation (allo-SCT) recipients versus 17% for non-recipients (P = 0.050). Multivariate analysis showed that the percentage of marrow blasts and achievement of MCyR or deeper could predict survival.
CONCLUSIONS: Outcomes of 3q26.2/MECOM-rearranged CML are poor despite the availability of multiple BCR::ABL1 tyrosine kinase inhibitors (TKIs). Third-generation TKIs in combination with novel agents and possible allo-SCT could be considered given the poor outcomes and resistance to second-generation TKIs.
METHODS: We reviewed consecutive adult patients with 3q26.2/MECOM-rearranged CML between January 1, 1998 and February 16, 2023. Rearrangements of 3q26.2/MECOM were confirmed by conventional cytogenetics, and fluorescence in situ hybridization starting in 2015.
RESULTS: We identified 55 patients with MECOM-rearranged CML, including 23 in chronic phase (CP) or accelerated phase (AP) and 32 in blast phase (BP). Nine patients (16%) achieved a major cytogenetic response (MCyR) or deeper. At a median follow-up of 89 months, median survival was 14 months. The 5-year survival rate was 19% overall, 23% in CML-CP/AP, and 15% in CML-BP. In the 6-month landmark analysis, the 5-year survival rate was 41% for allogeneic stem cell transplantation (allo-SCT) recipients versus 17% for non-recipients (P = 0.050). Multivariate analysis showed that the percentage of marrow blasts and achievement of MCyR or deeper could predict survival.
CONCLUSIONS: Outcomes of 3q26.2/MECOM-rearranged CML are poor despite the availability of multiple BCR::ABL1 tyrosine kinase inhibitors (TKIs). Third-generation TKIs in combination with novel agents and possible allo-SCT could be considered given the poor outcomes and resistance to second-generation TKIs.
摘要：
目的：评估3q26.2/MECOM重排慢性粒细胞白血病（CML）患者的预后。
方法：我们回顾了1998年1月1日至2023年2月16日期间3q26.2/MECOM重排CML的连续成年患者。3q26.2/MECOM的重排被常规细胞遗传学证实，和荧光原位杂交从2015年开始。
结果：我们确定了55例MECOM重排的CML患者，其中慢性期（CP）或加速期（AP）23例，爆炸期（BP）32例。9名患者（16％）达到了主要的细胞遗传学反应（MCyR）或更深。在中位随访89个月时，中位生存期为14个月.5年生存率为19％，23%的CML-CP/AP，和15%的CML-BP。在为期6个月的里程碑分析中，异基因干细胞移植(allo-SCT)受者的5年生存率为41%,非受者为17%(P=0.050).多因素分析表明，骨髓母细胞的百分比和MCyR或更深的成就可以预测生存率。
结论：尽管存在多种BCR::ABL1酪氨酸激酶抑制剂（TKIs），但3q26.2/MECOM重排的CML的预后较差。考虑到不良的结果和对第二代TKIs的抗性，可以考虑将第三代TKIs与新型药物和可能的allo-SCT结合使用。
方法：我们回顾了1998年1月1日至2023年2月16日期间3q26.2/MECOM重排CML的连续成年患者。3q26.2/MECOM的重排被常规细胞遗传学证实，和荧光原位杂交从2015年开始。
结果：我们确定了55例MECOM重排的CML患者，其中慢性期（CP）或加速期（AP）23例，爆炸期（BP）32例。9名患者（16％）达到了主要的细胞遗传学反应（MCyR）或更深。在中位随访89个月时，中位生存期为14个月.5年生存率为19％，23%的CML-CP/AP，和15%的CML-BP。在为期6个月的里程碑分析中，异基因干细胞移植(allo-SCT)受者的5年生存率为41%,非受者为17%(P=0.050).多因素分析表明，骨髓母细胞的百分比和MCyR或更深的成就可以预测生存率。
结论：尽管存在多种BCR::ABL1酪氨酸激酶抑制剂（TKIs），但3q26.2/MECOM重排的CML的预后较差。考虑到不良的结果和对第二代TKIs的抗性，可以考虑将第三代TKIs与新型药物和可能的allo-SCT结合使用。
