Hemoglobin Patterns in Patients with Sickle Cell Hemoglobinopathies

Abstract

Background: Hemoglobinopathy is a group of inherited disorders characterized by structural variations of the hemoglobin molecule; and sickle cell disease constitutes one of the major genetic blood disorders in Sudan. The aim of this study was to determine the hemoglobin patterns of patients with sickle cell hemoglobinopathies.

Methods: This is a hospital based case control study conducted at the Military Hospital, Omdurman. A total of 100 blood samples were collected, 70 cases diagnosed or suspected to have sickle cell disease and 30 was healthy control. Sickling test and hemoglobin (Hb) electrophoresis were performed using Capillary 2 Flex Piercing (SEBIA).

Results: Capillary Hb electrophoresis exposed the following variants of sickling hemoglobinopathies among cases group: 37 patients (52.9%) AS pattern, 1 patient (1.4%) AS/C Pattern, 8 patients (11.4%), S/B Thalassemia pattern, 1 patient (1.4%) S/C pattern, 3 patients (4.3%) S/D pattern and 20 patients (28.6%) SS pattern. While Hb electrophoresis obtained from normal healthy controls' group revealed normal expected values, the means of HbA (α2β2) was 95% with small amounts (<3.5%) of Hb A2 (α2δ2) and Hb F (α2γ2). The mean level of Hb was lower in patients' group while HbA2 demonstrated no significant change, while HbF and HbS showed different levels according to the type of hemoglobinopathy.

Conclusion: Different variants of sickle cell hemoglobinopathies were identified; AS patterns, AS/C patterns, S/B Thalassemia patterns, S/C patterns, S/D patterns and SS patterns were reported. Hemoglobin A2 had no significant difference in patients with sickle cell disease, while Hb F and Hb S showed significant elevation.