Homozygous Hereditary Elliptocytosis as the Cause of Haemolytic Anemia in Infancy

Two fatal cases of severe anaemia in infancy with ellpitocytosis in a Danish family are described. The severe progression of the disease is due to homozygosity for the gene for elliptocytosis. A statistical analysis of the pedigree shows linkage between the gene for elliptocytosis and the Rh‐genes. A morphological difference between the heterozygous and the homozygous elliptocytosis is shown to be that the longest diameter of the erythrocytes is significantly shorter in homozygotes than in heterozygotes. The similarity between the heredity in elliptocytosis, thalassaemia, and sickle cell anaemia is shown. Following splenectomy the haemolysis in propositus is compensated.