Hemoglobinopathies in Danish families

Based on cases referred for investigation, as well as a questionnaire sent to all medical and pediatric departments in Denmark, 48 cases of hemoglobinopathy in 15 families of Danish ancestry are reviewed. 18 Danes in six families have been identified as having beta-thalassemia, and remarkably one - a homozygote - has beta-thalassemia intermedia requiring treatment with iron-chelation therapy. A further 36 Danes in 9 families have a hemoglobin variant: five unstable hemoglobins (Volga, Niteroi, and three unidentified), one hereditary methemoglobinemia (M-Arhus), one polycythemia (Ty Gard) and 2 asymptomatic (Athens-Georgia and Hafnia). Although rare in Danish families, a hemoglobinopathy should be considered in families with an unexplained chronic hemolytic anemia, cyanosis or polycythemia.