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Future studies aimed at better defining the optimal treatment of these patients are warranted. ""Patients with sickle cell disease (SCD) from the Southwestern (SW) Province of Saudi Arabia have variable fetal hemoglobin (HbF) levels and have HBB gene cluster haplotypes of African origin. We studied 77 patients, aged 17.7 �� 10 (range 4�C46) years (69% HbS homozygotes and 31% HbS-��0 thalassemia), to determine the associations of known HbF quantitative trait loci (QTL) with HbF concentration. HBB gene cluster haplotypes were 74% Benin, 22% Bantu, and 4% others. Genotyping Single nucleotide polymorphism (SNPs) in BCL11A, HBS1L-MYB, and OR51B5/6 showed that BCL11A was the sole QTL associated with HbF level. We compared these findings with two studies of African American http://www.selleckchem.com/products/epz-5676.html with SCD. After adjusting for the BCL11A genotype, Saudi cases from the SW Province had HbF levels almost twice that of African Americans http://www.selleck.cn/products/Staurosporine.html (P http://www.selleckchem.com/products/ly2109761.html disease (SCD). High HbF levels decrease the incidence and severity of vaso-occlusive acute painful episodes, acute chest syndrome, and osteonecrosis [1]. Moreover, HbF is associated with increased survival [2]. HbF levels vary among patients with SCD and its expression is influenced by genetic [3, 4] and epigenetic factors [5, 6]. Three quantitative trait loci (QTL) play a major role in determining HbF expression and account for about 30% of the variability in HbF [7]. BCL11A and HBS1L-MYB are associated with HbF expression in African Americans with SCD [8, 9]. In addition, a region on chromosome 11 that harbors olfactory receptor gene cluster was recently shown to be a potential modifier of HBG expression in SCD [10]. SCD is common in Saudi Arabia with the highest incidence in the Eastern and Southwestern (SW) provinces [11, 12]. Patients from the SW Province have many complications of this disease and have phenotypic differences from African Americans that include a high prevalence of splenomegaly, rare CNS disease, and absence of leg ulcers [13�C15]. In contrast, Eastern Province patients have a milder phenotype that is related to a nearly uniformly high HbF level that is associated with the Saudi�CIndian haplotype of the HBB gene-like cluster [16].