The FA-characteristic 3q gains nearly always harbor one of the most aggressive leukemogenic oncogenes, EVI1, which was first detected to be amplified and overexpressed as an initial event in FA-derived AML transformation in patient material and cell lines from a patient with biallelic FANCD1/BRCA2 mutations [16, 31, 38] and subsequently shown to result in overexpression of EVI1 [37]. The gene discussed is MECOM; the disease is Friedreich ataxia.