The WHO’s 2016 revision of the classification of myeloid neoplasms and acute leukemias [155] introduced a new category of diseases defined as myeloid neoplasms with germline predisposition and pre-existing platelet disorders, that includes those that evolve with molecular variants on ankyrin repeat domain 26 gene (ANKRD26) or on transcription factors ETV6 and RUNX1 [18,19,20,22,122,156,157]. The gene discussed is ANKRD26; the disease is acute leukemia.