Frequency of Sickle Cell Trait among Relatives of Sickle Cell Anemia Patients in Al-Gadaref State-Sudan

Abstract

Background: The term "sickle cell disease" refers to a collection of autosomal recessive genetic disorders characterized by the Hb S variant of the B-globin gene. Sickle cell disease is a major public health concern that has a great impact on both individuals and societies. In Sudan, sickle cell anemia is one of the major types of anemia, especially western Sudan, where the sickle cell gene is frequent, so this study aimed to determine the frequency of sickle cell trait (HbAS) among relatives of sickle cell anemia patients (HbSS) in Al-Gadaref state –Sudan.

Methodology: A descriptive, cross-sectional, analytical study was carried out for seventeen  families with one hundred and fourteen individuals with different ethnic descents. 56 Males  and 58 Females, age ranged between 1 -70 years compared with 30 healthy individuals  age ranged between 28-73 as a control group.  Venous blood (2.5ml) was collected from each individual in an ethylene diamine tetra acetic acid (EDTA) container for  complete blood count (CBC), erythrocyte sedimentation rate (ESR), sickling test, and hemoglobin electrophoresis.

Results: The data showed that (67%) of the study population were positive and (33%) were negative for sickling test, the hemoglobin electrophoresis showed high frequency of (HbAS) (66.7%), normal people (HbAA) (24.6%), HbSS (5.3%), hemoglobin C trait (HbAC) (1.8%), and sickle cell with hemoglobin C disease (HbSC) (1.8%). The mean of Hb level, TRBCs, and PCV in patients with HbSS and HbSC were lower than in HbAS and HbAC. The MCV, MCHC, and MCH, showed no significant difference between different groups. The total leukocytes, was significantly elevated in HbSS and HbSC. Platelets were higher in HbSS and lower in HbSC, and ESR was elevated in both as compared with other groups.

Conclusion: The sickle cell trait is highly frequent among the relatives of sickle cell anemia patients and the spreading degree could be due to the high degree of consanguineous marriage in the studied population.