The 5th edition of WHO classification of hematolymphoid tumors categorized myeloid neoplasms with germline predisposition into three subgroups: myeloid neoplasms with germline predisposition without a preexisting platelet disorder or organ dysfunction (DDX41, TP53, CEBPA); myeloid neoplasms with germline predisposition and pre-existing platelet disorder (RUNX1, ANKRD26, ETV6); and myeloid neoplasms with germline predisposition and potential organ dysfunction (GATA2, Shwachman-Diamond syndrome (SDS) [152], Fanconi anemia (FA) [153], Down syndrome [154], etc.)[2,155]. The gene discussed is GATA2; the disease is myeloid neoplasm.