Germline monoallelic variants in DDX41, often frame-shift variants in the N-terminal region (Quesada et al. 2019), have been associated with an increased risk of myeloid malignancies, including MDS and AML (Lewinsohn et al. 2016; Polprasert et al. 2015). The gene discussed is DDX41; the disease is acute myeloid leukemia.