Mediterranean Journal of Hematology and Infectious Diseases (Aug 2014)
Haematological values in Homozygous Sickle Cell Disease in Steady State and Haemoglobin Phenotypes AA Controls in Lagos, Nigeria.
Abstract
Background: Sickle cell disease is a genetic abnormality involving the haemoglobin. The consequent haemoglobin S causes polymerization of haemoglobin resulting in haemolysis and anaemia. Although, it is primarily a red cell disorders, the white blood cells and platelets are also affected by the mutation. The study aimed 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. A blood sample of 4.5mls 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 subjects and 98 controls were enrolled. Cases consisted of 57 (55.33%) females and 46 (44.66%) males, while controls were made up of 68 (69.38%) females and 30 (30.61%) males. The overall mean haemoglobin concentration, packed cell volume, mean cell volume, mean cell haemoglobin and mean cell haemoglobin concentration of the cases were 7.93±1.47, 24.44±4.68, 81.52±7.89, 26.50±3.20, 32.50±1.07 respectively, for the controls were 13.83±1.32, 43.07±3.95, 86.90±4.69, 28.50±1.34,32.06±0.90 respectively. The overall mean of white blood cell and platelet counts for the cases were 10.27±3.94 and 412.71±145.09 respectively and for the controls 5.67±1.59 and 222.82±57.62. 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.
