Indeed, as reported by the 2016 WHO revision [2], in up to one-third of cases, MDS/MPN with neutrophilia is characterized by the presence of recurrent mutations in ETNK1 (3.7–13.3%) [61,62,63] and SETBP1 (7.4–48%) [64,65,66] genes, often in association with ASXL1 (20–81%), whose presence has been linked to a more aggressive disease [4,36,43,58,67,68]. Here, SETBP1 is linked to myeloproliferative neoplasm.