The “myelodysplastic syndrome/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T)” in the 2016 WHO classification [2] is renamed MDS/MPN with SF3B1 mutation and thrombocytosis (MDS/MPN-T) in the WHO 2022 classification [3], and split into two entities in the ICC [4], represented by MDS/MPN with thrombocytosis and SF3B1 mutation (MDS/MPN-T SF3B1), and MDS/MPN with ring sideroblasts and thrombocytosis, not otherwise specified, in the absence of SF3B1 mutation (MDS/MPN RS-T, NOS), as reported in Table 1. Here, SF3B1 is linked to intrahepatic cholangiocarcinoma.