Outcome of 3q26.2/MECOM rearrangements in chronic myeloid leukemia.

IF 1.7 4区医学 Q3 HEMATOLOGY International Journal of Hematology Pub Date : 2024-08-01 Epub Date: 2024-05-15 DOI:10.1007/s12185-024-03787-z

Hiroki Akiyama, Hagop Kantarjian, Elias Jabbour, Ghayas Issa, Fadi G Haddad, Nicholas J Short, Shimin Hu, Jo Ishizawa, Michael Andreeff, Koji Sasaki

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Abstract

Study aims: 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.

Conclusion: 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.

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慢性髓性白血病中 3q26.2/MECOM 基因重排的结果

研究目的评估3q26.2/MECOM重组慢性髓性白血病（CML）患者的预后：我们回顾了1998年1月1日至2023年2月16日期间连续收治的3q26.2/MECOM重排CML成人患者。3q26.2/MECOM重排由常规细胞遗传学和2015年开始的荧光原位杂交证实：我们发现了 55 例 MECOM 重排 CML 患者，其中 23 例处于慢性期（CP）或加速期（AP），32 例处于爆发期（BP）。9名患者（16%）获得了主要细胞遗传学反应（MCyR）或更深。中位随访时间为 89 个月，中位生存期为 14 个月。总体5年生存率为19%，CML-CP/AP为23%，CML-BP为15%。在6个月的地标分析中，异基因干细胞移植（allo-SCT）受者的5年生存率为41%，而非受者为17%（P = 0.050）。多变量分析表明，骨髓爆破的百分比和达到MCyR或更深可预测存活率：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CML的治疗效果不佳。鉴于第二代 TKIs 的不良预后和耐药性，可以考虑将第三代 TKIs 与新型药物联合使用，并可能进行异体 SCT。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

International Journal of Hematology 医学-血液学

CiteScore

3.90

自引率

4.80%

发文量

223

审稿时长

6 months

期刊介绍： The International Journal of Hematology, the official journal of the Japanese Society of Hematology, has a long history of publishing leading research in hematology. The journal comprises articles that contribute to progress in research not only in basic hematology but also in clinical hematology, aiming to cover all aspects of this field, namely, erythrocytes, leukocytes and hematopoiesis, hemostasis, thrombosis and vascular biology, hematological malignancies, transplantation, and cell therapy. The expanded [Progress in Hematology] section integrates such relevant fields as the cell biology of stem cells and cancer cells, and clinical research in inflammation, cancer, and thrombosis. Reports on results of clinical trials are also included, thus contributing to the aim of fostering communication among researchers in the growing field of modern hematology. The journal provides the best of up-to-date information on modern hematology, presenting readers with high-impact, original work focusing on pivotal issues.