%0 Journal Article
%T Outcome of 3q26.2/MECOM rearrangements in chronic myeloid leukemia.
%A Akiyama H
%A Kantarjian H
%A Jabbour E
%A Issa G
%A Haddad FG
%A Short NJ
%A Hu S
%A Ishizawa J
%A Andreeff M
%A Sasaki K
%J Int J Hematol
%V 120
%N 2
%D 2024 Aug 15
%M 38748089
%F 2.319
%R 10.1007/s12185-024-03787-z
%X <B>OBJECTIVE: </B>To evaluate the outcomes of patients with 3q26.2/MECOM-rearranged chronic myeloid leukemia (CML).<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>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.