Abstract
BackgroundSickle 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.MethodsA 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.ResultsA 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 *103/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.ConclusionHomozygous 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.
Highlights
Sickle cell disease is a genetic abnormality involving the haemoglobin
A case–control study was conducted amongst Sickle cell patients in steady state attending the sickle cell clinics of Lagos State University Teaching Hospital Ikeja (LASUTH) referred from private clinics outside the hospital and other clinics in the hospital and whose diagnoses were confirmed by alkaline haemoglobin electrophoresis and controls consisting of medical students, doctors and nurses of the institution between September to December 2011 after obtaining approval from LASUTH, the institution’s Ethics and Research Committee
The red cell indices were generally lower in sickle cell patients than controls with haemoglobin phenotype AA while the white blood cells and platelet counts were higher than control values
Summary
Sickle cell disease is a genetic abnormality involving the haemoglobin It is primarily a red cell disorders, the white blood cells and platelets are affected by the mutation. The consequent haemoglobin S causes polymerization of haemoglobin resulting in haemolysis and anaemia. Haemoglobin S resulting from the substitution causes polymerization of haemoglobin and red cell sickling on exposure to low oxygen tension and unsickle on oxygenation. The repeated sickling and unsickling damages the red cell membrane leading to irreversibly sickled red cell even when the oxygen pressure is increased reducing red cell life span as a result of membrane damage inducing anaemia. Haemolysis consequent to the damaged red cell membrane could be intravascular or extravascular The former results from the lysis of complement-sensitive red cells [1] and haemoglobin lost during sickling-induced membrane damaged. Increased susceptibility to mechanically induced cell fragmentation has been documented in-vitro and in sickle cell patients undergoing vigorous exercise [3]
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