Predominantly hemizygous, predicted loss-of-function mutations have been described at low frequency in myeloid malignancies, including myeloproliferative neoplasms (MPNs), myelodysplasia (MDS), and AML (Ernst et al., 2010; Makishima et al., 2010; Nikoloski et al., 2010; Ley et al., 2013), and Ezh2 loss in mouse models has been shown to lead to the development of multiple long-latency hematological malignancies, predominantly MDS, MPN, and T-adult lymphoblastic leukemia/T cell lymphoma (Simon et al., 2012; Mochizuki-Kashio et al., 2015). Here, EZH2 is linked to acute myeloid leukemia.