Sickle cell disease in Middle East Arab countries

Area of Science:

• Hematology
• Genetics
• Epidemiology

Background:

• The sickle cell (HbS) gene is a significant genetic factor in Middle Eastern Arab countries, contributing to blood genetic disorders.
• Malaria endemicity is a key factor in the high prevalence and persistence of HbS and thalassaemia genes in the region.

Purpose of the Study:

• To review the frequency, distribution, and clinical features of the HbS gene in Middle Eastern Arab countries.
• To outline the management, prevention, and gene interactions of HbS with other hemoglobinopathies in the region.

Main Methods:

• Review of existing literature on HbS gene frequency, distribution, and haplotypes in the Middle East.
• Analysis of β-globin gene haplotypes associated with HbS and their correlation with clinical phenotypes of sickle cell disease (SCD).

Main Results:

• HbS gene frequency varies across Middle Eastern Arab countries, with evidence of multiple origins.
• Two distinct clinical forms of SCD (benign and severe) are associated with the Saudi-Indian and Benin β-globin gene haplotypes, respectively.
• HbS is predominantly linked to the Saudi-Indian haplotype in most of these countries.

Conclusions:

• The Saudi-Indian and Benin haplotypes play a crucial role in determining the clinical severity of sickle cell disease in the Middle East.
• Understanding HbS gene interactions and distribution is vital for effective management and prevention strategies in the region.