Abstract

 Background and Objectives

 Hemoglobinopathies are the most common single-gene disorders in Iran. Sistan and Balouchistan has 2300 hemoglobinopathy patients who judiciously receive blood in the southeast of Iran. In this study, the prevalence rate of hemoglobinopathies was evaluated in the province.

 Materials and Methods

 This descriptive analytical study was performed on 2129 people at the age range of 5-10 years who were selected by multistage cluster sampling method. The number of samples in each city was proportional to its population size. Hematologic parameters were evaluated by automated cell counter on EDTA whole blood, hemoglobin A2 (HbA2) was determined by column chromatography, and hemoglobins were separated on Acetate Cellulose at pH= 8.6. The samples with abnormal hemoglobins underwent electrophoresis at pH= 6.1.

 Results

  The results indicated 9 . 7% affected with minor thalassemia, with the frequency being variable between 6.2% in north cities to 12.9% in south cities of the province. A total of 0.9, 1.7 and 0.1% were heterozygous for hemoglobin S, D, and C, respectively. HbA2 level of 72.4% of heterozygous hemoglobinopathies was estimated to be within the range of healthy individuals.

 Conclusions

  The prevalence rate of β-thalassemia gene in the southern region of the province was higher than the average national rate. Current pre-marriage screening protocol is not able to identify all hemoglobinopathies in heterozygous cases. It is recommended where hemoglobinopathies are common, hemoglobin S detection be included in the screening program.