Abstract
The Hb S gene is widely distributed in Saudi Arabia with a prevalence of 5% in parts of the Western (Red Sea) area and 24% in parts of the Eastern (Gulf) region. We compared hematological data of Hb SS patients from Jeddah (west) and Dammam (east).
Compared to Jeddah and American patients, Dammam patients were less anemic, more microcytic, had higher HbF levels. Most retained splenic function. We speculate that the Hb S gene may have entered the southwestern Arabian peninsula from Africa in antiquity. As it progressed northeasterly it interacted with other endemic, genetic traits in isolated populations (α thal, h HPFH, etc.). These interactions ameliorated HbSS disease reducing hemozygous lethality. Reproduction by homozygotes, a high rate of consanguinity and the selective advantage of endemic falciparum malaria could have combined to produce the high prevalence of the HbS gene found in the eastern regions today.
These preliminary studies indicate a wide and unique variability of sickle cell hemoglobinopathies of the Arabian Peninsula.
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Al-Awamy, B., Zamachari, F., Pearson, H. et al. 877 SICKLE CELL ANEMIA IN SAUDI ARABIA: SPECULATIONS ABOUT REGIONAL VARIABILITY IN FREQUENCY AND SEVERITY. Pediatr Res 19, 257 (1985). https://doi.org/10.1203/00006450-198504000-00907
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DOI: https://doi.org/10.1203/00006450-198504000-00907