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

• DOI: 10.1007/s12185-024-03787-z
• Publication Date: 2024-05-15T12:02:04Z
• Authors: Hiroki Akiyama; Hagop Kantarjian; Elias Jabbour; Ghayas Issa; Fadi G. Haddad; Nicholas J. Short; Shimin Hu; Jo Ishizawa; Michael Andreeff; Koji Sasaki
• Keywords: Adult; Male; Gene Rearrangement; Adolescent; Middle Aged; Survival Rate; Young Adult; Treatment Outcome; Leukemia, Myelogenous, Chronic, BCR-ABL Positive; Humans; Female; Chromosomes, Human, Pair 3; Aged; Follow-Up Studies; Retrospective Studies

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.