In 2010, SETBP1 mutations were identified as causative in the rare,lethal disorder Schinzel-Giedion syndrome (8).Recently, SETBP1 mutations have also been identified in aCML and otherclosely related hematological malignancies, including CNL, CMML, unclassified MDS, MPNs,and secondary AML evolving from MDS at variable frequencies (1.7-25%) (9-13), asshown in Table 1. This evidence concerns the gene SETBP1 and chronic myelomonocytic leukemia.