Hemoglobinopathies in Turkey.

214 Turkey is settled at a crossroads of the earth’s continents, so migrations throughout the world, especially between Asia, Europe and Africa, have affected Turkey in numerous ways, including in the evolution of hemoglobin disorders. In the middle of the 1940s, many thalassemic patients and abnormal hemoglobins began to be diagnosed at Istanbul University, and the pioneering works of Prof. Aksoy in the second half of the last century were of great significance in this field. Investigations into the hemoglobinopathies and thalassemias have been performed in many different areas of Turkey and are continuing, but most of these surveys are restricted to local regions. These studies have pointed out that these disorders are especially cumulated in the central southern part of Turkey. Thalassemia surveys were pioneered by Aksoy et al. . Arcasoy et al. found the β-thalassemia incidence as 2.1% in a healthy population in 1978 . In accordance with the data given by the Ministry of Health and by Altay [5] (cumulated from local survey results of different areas of Turkey between 2000-2006), the incidence of β-thalassemia and abnormal hemoglobins has been reported as approximately 4.3%, ranging from 0.6% to 13.0% in different surveys . The incidence surveys are shown in Table 1. The surveys investigating incidences and frequencies were done in different populations, some in cord blood samples, some in premarital screening procedures and some in selected areas known to have high incidence of hemoglobinopathies and thalassemia. In screening programs, attention must be given to the population selection. Screening of hospital records or calculation of percentages of cumulated patient populations should not be used for prevalence/incidence surveys or reports to avoid confusion. Premarital screening programs could be useful in the beginning for identifying couples at risk of having affected children.