In contrast to other genes with strong predisposition to familial myeloid neoplasms, such as GATA2, RUNX1, or CEBPA, which are frequently mutated in sporadic MDS/AML cases, somatic DDX41 variants are exceedingly rare (0.4%) in the absence of predisposing germline DDX41 variants and were reported only in five out of 1385 unselected patients with MDS or AML [27]. Here, CEBPA is linked to acute myeloid leukemia.