Examples for autosomal recessive inherited diseases with high MDS/AML risk are classical bone marrow failure syndromes such as Fanconi anemia (FA) and Shwachman-Diamond syndrome, examples for X-linked inheritance are some forms of dyskeratosis congenita (DKC1) or FA (FANCB) and the immunodeficiency Wiskott-Aldrich syndrome. Here, DKC1 is linked to Friedreich ataxia.