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Causative germline variants in the <jats:italic>DDX41<\/jats:italic> predispose to the development of myeloid neoplasms (MNs), especially myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Almost 3%\u20135% of all patients with MDS or AML carry a pathogenic or likely pathogenic germline <jats:italic>DDX41<\/jats:italic> variant, while half of them acquire a somatic second hit in the other allele. <jats:italic>DDX41<\/jats:italic>\u2010associated MNs exhibit unique clinical characteristics compared to other hematological malignancies with germline predisposition: MNs occur mostly at advanced age and follow an indolent clinical course. Male carriers are more prone to develop MDS or AML than females. <jats:italic>DDX41<\/jats:italic>\u2010associated MN is often hypoplastic, and the malignancy may be preceded by cytopenias.<\/jats:p>","DOI":"10.1002\/hem3.145","type":"journal-article","created":{"date-parts":[[2024,8,13]],"date-time":"2024-08-13T10:33:55Z","timestamp":1723545235000},"source":"Crossref","is-referenced-by-count":14,"title":["How to manage patients with germline <i>DDX41<\/i> variants: Recommendations from the Nordic working group on germline predisposition for myeloid neoplasms"],"prefix":"10.1002","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5634-7156","authenticated-orcid":false,"given":"Panagiotis","family":"Baliakas","sequence":"first","affiliation":[{"name":"Department of Immunology, Genetics and Pathology Uppsala University  Uppsala 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