Evaluation of Hemoglobin Variants among Patients with Sickle Cell Anemia in Khartoum State, Sudan

Abstract

Mohammed Osman Ali Mohammed, Hadeel Ali, Ibrahim Bakhit and Ghanem Mohammed Mahjaf

Background: Sickle cell anemia (SCA) is one of the most common inherited hemoglobin disorders in Sudan, with a particularly high prevalence among populations originating from western regions of the country. The disease is characterized by abnormal hemoglobin S (HbS), which leads to chronic hemolytic anemia and recurrent vaso-occlusive complications. Assessment of hemoglobin variants is essential for disease diagnosis, monitoring, and evaluating treatment response, particularly to hydroxyurea therapy.

Methods: A hospital-based case-control study was conducted between November 2022 and March 2023 at the Sickle Cell Clinic of Gaafar Ibn Auf Specialized Hospital and Omdurman Children's Emergency Hospital, Khartoum State, Sudan. A total of 75 participants were enrolled, including 50 patients diagnosed with sickle cell anemia and 25 healthy controls. Venous blood samples were collected in EDTA tubes, and hemoglobin variants (HbA, HbA2, HbS, and HbF) were measured using the CAPILLARYS 2 FLEX PIERCING system. Statistical analysis was performed using SPSS software, with a p-value <0.05 considered statistically significant.

Results: The mean percentages of HbS, HbA, HbA2, and HbF among patients with SCA were 78%, 7.2%, 3.0%, and 10.0%, respectively, compared with 0.0%, 97.0%, 2.5%, and 0.0% among healthy controls. Significant differences were observed between cases and controls in HbS, HbA, and HbF levels (p <0.001), whereas HbA2 levels did not differ significantly (p =0.40). Among patients receiving hydroxyurea therapy, the mean HbF level was markedly higher (14%) than among those not receiving hydroxyurea (1%) (p <0.001). No significant associations were found between hemoglobin variants and patients' age or sex.

Conclusion: Patients with sickle cell anemia demonstrated significantly elevated HbS and HbF levels and reduced HbA levels compared with healthy individuals. Hydroxyurea therapy was associated with a substantial increase in fetal hemoglobin (HbF), supporting its beneficial role in the management of sickle cell anemia.

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