EVI1high was not correlated with age, sex, or white blood cells (WBC), whereas patients with EVI1high had a significantly higher frequency of (1) acute megakaryoblastic leukemia (FAB-M7) (23.7% vs. 6.0%, p = 0.001) (2), MLL rearrangements (39.5% vs. 14.4%, p < 0.0001), especially MLL-AF9 (15.8% vs. 6.8%, p = 0.046) (3); unfavorable cytogenetic aberrance (55.3% vs. 24.8%, p < 0.0001), but only one patient harbored a 3q26 rearrangement. Here, KMT2A is linked to acute megakaryoblastic leukemia.