Congenital dyserythropoietic anaemias (CDA) are disorders with defective production of red blood cells (RBC) and mild haemolysis. CDAs are transmitted by recessive inheritance. There are several different clinical forms of CDA leading to anaemia, yellow skin (jaundice), enlargement of liver and spleen (hepatosplenomegaly) and iron overload. CDA I may be associated with skeletal dysmorphies features, particularly affecting the digits. The prevalence does not exceed 1/100.000 births. Interferon-alpha is an effective treatment in severe cases. Iron overload may necessitate treatment by iron depletion.

The diagnosis relies on morphologic examination of bone marrow and sequenzing of the CDANI-gene.