Hemoglobin F modulation in sickle cell disease: Experience in a single center in Iraqi Kurdistan

Abstract

Background: Sickle cell disease (SCD) constitutes an important health problem in the Eastern Mediterranean countries including Iraq. Previous studies from northern Iraq had focused on various aspects of these hemoglobinopathies, but none focused on response of these patients to hemoglobin F modulating agents. Patients and Methods: In this prospective study, a total of 106 patients with SCD were enrolled. They were aged 5–56 years (median 19.2 years) with a male to female ratio of 2.1:1.0. The patients were initiated on hydroxycarbamide and evaluated clinically and hematologically for response prior to therapy, at 2 and 6 months post-therapy, and at last follow-up. Results: Out of the 106 patients enrolled, 100 (94%) patients had a significant response. The responders showed a significant increase in their total hemoglobin (Hb), mean corpuscular volume (MCV), and fetal hemoglobin (HbF), reduction in their blood requirement, and vaso-occlusive crises. The therapy was quite well tolerated and there were very limited number of side effects. After a median follow-up of 4 years and 7 months (range 1–9 years), those responders at 6 months continued to do so, with 72% remaining transfusion free at their last follow-up. Conclusion: The remarkable response to hydroxycarbamide in the enrolled patients is consistent with other studies worldwide, and thereby hydroxycarbamide offers itself as a cost-effective first-line management option in SCD patients in developing countries.

Keywords

• Duhok
• hydroxyurea
• Iraq
• Kurds
• sickle cell disease