Abstract

From the information obtained from 374 patients diagnosed in Hacettepe Children’s Hospital during the years 1975 - 1985, and from the surveys and publications made by other authors, the distribution of hemoglobinopathies in Turkey has been determined.

Population studies indicate that Hb S is present only in Çukurova, Manavgat and İstanbul. Beta thalassemia has been found to be present all over Turkey but most frequently in west and southwest Turkey.

The number of patients with β -thalassemia major was also found to be higher in certain areas such as Çukurova, Ankara, Konya and western Anatolia.

Detailed hematological examination of the patients with β -thalassemia intermedia revealed the presence of at least seven different mild β -thalassemia determinants.

It has been reported that 19 abnormal hemoglobin variants are to be found in Turkey but in some isolated areas the only common ones are Hb S and Hb E.

The number of patients actually diagnosed as having β -thalassemia major was much lower than the expected figure arrived at by calculation. The reasons for the discrepancy in these figures are discussed. The importance of prenatal diagnosis in the eradication of hemoglobinopathies is stressed.

Keywords: abnormal hemoglobin, hemoglobinopathy, sickle cell anemia, thalassemia

How to cite

1.
Altay Ç, Gürgey A. Distribution of hemoglobinopathies in Turkey Based on studies conducted at Hacettepe Children’s Hospital and reviews of other studies. Turk J Pediatr 1986; 28: 219-229. https://doi.org/10.24953/turkjpediatr.1986.3958