Department of Haematology and Blood transfusion, University of Lagos, P.M.B 12003, Surulere, Lagos, Nigeria; National Sickle Cell Centre, Surulere, Lagos, Nigeria
Adeyemo, T., Department of Haematology and Blood transfusion, University of Lagos, P.M.B 12003, Surulere, Lagos, Nigeria; Ojewunmi, O., National Sickle Cell Centre, Surulere, Lagos, Nigeria; Oyetunji, A., Department of Haematology and Blood transfusion, University of Lagos, P.M.B 12003, Surulere, Lagos, Nigeria
Introduction: Sickle cell disease (SCD) is the most common inherited disorder of haemoglobin worldwide. This study evaluated the chromatographic patterns and red blood cell indices of sickle cell patients to determine the co-inheritance of other haemoglobin (Hb) variants and -thalassaemia trait in SCD patients in Nigeria. Methods: Red cell indices, blood film, sickle solubility test, Hb electrophoresis using alkaline cellulose acetate membrane, and chromatographic patterns using Bio Rad HPLC Variant II were evaluated for 180 subjects. Results: Based on low MCV<76fL and MCH<25 pg, in the presence of elevated A2 >4.0% on HPLC and Hb variants eluting outside the S and C windows, at least four haemoglobin phenotypes (SS: 87.7%; SC: 1.1%; SD Punjab: 0.6%; Sβ-thalassemia: 10.6%) were identified. Mean Hb F% was 8.1±5.1 (median 7.65) for Hb SS and 6.03±5.2 (median 3.9) for Hb Sβ-thalassemia trait. Majority of Hb SS (69.1%) had Hb F% less than 10 while 27.6% had 10-19.9 and 3.2% had ≥ 20. Mean Hb F% was higher in female Hb SS (9.55±5.09; mean age 7.4±3.8 years) than the males (7.63±4.80; mean age 6.9±3.8 years) (P=0.02). A borderline significant negative correlation between age and Hb F levels among Hb SS subjects (r= -0.169 P=0.038) was also observed. Conclusion: Our data suggests that α and β- thalassaemia traits, and other haemoglobin variants co-exist frequently with SCD in our population. © Titilope Adeyemo et al.
hemoglobin F; hemoglobin beta chain; hemoglobin D Punjab; hemoglobin F; hemoglobin variant; adolescent; alpha thalassemia; article; child; controlled study; female; hemoglobin SD disease; high performance liquid chromatography; human; major clinical study; male; mean corpuscular hemoglobin; mean corpuscular volume; Nigeria; prevalence; prospective study; sex difference; sickle cell beta thalassemia; Anemia, Sickle Cell; beta-Thalassemia; blood; clinical trial; comorbidity; developing country; genetics; hemoglobin determination; heterozygote; preschool child; procedures; sickle cell trait; Adolescent; Anemia, Sickle Cell; beta-Globins; beta-Thalassemia; Child; Child, Preschool; Chromatography, High Pressure Liquid; Comorbidity; Developing Countries; Erythrocyte Indices; Female; Fetal Hemoglobin; Hemoglobinometry; Hemoglobins, Abnormal; Heterozygote; Humans; Male; Nigeria; Prevalence; Prospective Studies; Sickle Cell Trait