A distal enhancer of the GATA2 gene arising upon chromosomal 3q rearrangement ectopically activates EVI1 (ecotropic virus integration site-1) expression, which leads to the concomitant loss of GATA2 transcription.40 The loss of GATA2 is associated with acute myeloid leukemia (AML), and GATA2 haploid in sufficiency might provide the precise context for EVI1-mediated oncogenic transformation40,72,73 (Table 1). Here, GATA2 is linked to acute myeloid leukemia.