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Objective: To describe the effect of Hydroxyurea (HU) treatment in Omani Sickle Cell Disease (SCD) patients with different beta-globin gene cluster haplotypes. Materials and methods: A total of 52 cases treated with HU were enrolled in this study. Response to the drug was compared between patients with and without the XmnI polymorphism in the different betaglobin gene cluster haplotypes. We have classified our cohort into three categories: good responders to HU for those patients who had no crises and no hospitalization after 6 months of treatment; partial responders for those who had a reduction in the number of crises after the same period and non responders for those that remained clinically unchanged even after doubling the HU prescription. Results: Most patients homozygous or heterozygous for the Xmn I polymorphism (T/T or T/C) had higher levels of HbF prior treatment than those having the CC genotype and were classified under good or partial responders. Conclusions: Being the Xmn-I polymorphism associated with the haplotypes frequent in Oman and acting as enhancer of the already elevated HbF expression, HU treatment can be prospectively applied to predict responsiveness and treatment can be given to those with low HbF expression for beneficial lowering of cellular adhesion. HU treatment can ameliorate the clinical phenotype of the large majority of Omani patients with SCD.

Association of XmnI (-158 γG) Polymorphism and Response to Hydroxyurea in Omani S/S and S/β Patients