The new WHO classification introduced four broad categories of AML with germline predisposition: 1-nMyeloid neoplasms with germline predisposition without a pre-existing disorder or organ dysfunction, it includes AML with CEBPA and DDX41 mutations 2- Myeloid neoplasms with germline predisposition and pre-existing platelet disorders, which encompass RUNX1, ANKRD26 and ETV6 mutations 3- Myeloid neoplasms with germline GATA2 mutation and 4- Myeloid neoplasms with germline predisposition associated with inherited bone failure syndromes and telomere biology disorders. Here, DDX41 is linked to myeloid neoplasm.