Department of Leukemia, The University of Texas, MD Anderson Cancer Center, 1515 Holcombe Blvd. Unit 428, Houston, TX, 77030, USA.
Department of Hematopathology, The University of Texas, MD Anderson Cancer Center, Houston, TX, 77030, USA.
Int J Hematol. 2024 Aug;120(2):203-211. doi: 10.1007/s12185-024-03787-z. Epub 2024 May 15.
To evaluate the outcomes of patients with 3q26.2/MECOM-rearranged chronic myeloid leukemia (CML).
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.
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.
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.
评估 3q26.2/MECOM 重排慢性髓系白血病（CML）患者的结局。
我们回顾了 1998 年 1 月 1 日至 2023 年 2 月 16 日连续收治的 3q26.2/MECOM 重排的成年 CML 患者。3q26.2/MECOM 的重排在 2015 年开始通过常规细胞遗传学和荧光原位杂交来确认。
我们确定了 55 例 MECOM 重排的 CML 患者，其中 23 例处于慢性期（CP）或加速期（AP），32 例处于急变期（BP）。9 例（16%）患者获得主要细胞遗传学缓解（MCyR）或更深缓解。中位随访 89 个月时，中位生存期为 14 个月。总体 5 年生存率为 19%，CP/AP 期为 23%，BP 期为 15%。在 6 个月的时间点分析中，allo-SCT 组的 5 年生存率为 41%，而非 allo-SCT 组为 17%（P=0.050）。多变量分析表明，骨髓原始细胞百分比和获得 MCyR 或更深缓解可预测生存。
尽管有多种 BCR::ABL1 酪氨酸激酶抑制剂（TKI）可用，但 3q26.2/MECOM 重排 CML 的结局仍然很差。鉴于第二代 TKI 耐药且预后较差，可考虑使用第三代 TKI 联合新型药物和可能的 allo-SCT。
