Haematological values in homozygous sickle cell disease in steady state and haemoglobin phenotypes AA controls in Lagos, Nigeria

摘要

Background Sickle cell disease is a genetic abnormality involving the haemoglobin. Although, it is primarily a red cell disorders, the white blood cells and platelets are also affected by the mutation. The consequent haemoglobin S causes polymerization of haemoglobin resulting in haemolysis and anaemia. This study aims to provide baseline haematological values in sickle cell disease patients in steady state and compare the deviation from haemoglobin phenotype AA control values. Methods A case–control study was conducted amongst homozygous sickle cell patients attending the sickle cell clinics of Lagos State University Teaching Hospital Ikeja and haemoglobin phenotype AA controls. About 4.5mls of blood sample was collected from each participant for full blood count analysis. All blood samples were screened for HIV and haemoglobin phenotypes confirmed using cellulose acetate haemoglobin electrophoresis at pH 8.6. Results A total of 103 cases and 98 controls were enrolled. The overall mean haemoglobin concentration for cases was 7.93?±?1.47?g/dl, packed cell volume 24.44?±?4.68%, mean cell volume 81.52?±?7.89?fl, and mean cell haemoglobin 26.50?±?3.20?pg. While for controls, mean haemoglobin concentration was 13.83?±?1.32?g/dl, packed cell volume 43.07?±?3.95%, mean cell volume 86.90?±?4.69?fl, and mean cell haemoglobin 28.50?±?1.34?pg. The overall mean white blood cell counts for the cases was 10.27?±?3.94 *10^{3/μl and platelet counts of 412.71?±?145.09*103/μl. While white blood cell count for the controls was 5.67?±?1.59*103/μl and platelet counts of 222.82?±?57.62*103/μl. Conclusion Homozygous sickle cell disease patients have lower values of red cell parameters, but higher values of white cell and platelets counts compared to haemoglobin phenotype AA controls.}