ASSESSMENT OF THE RELATIONSHIP BETWEEN FOETAL HAEMOGLOBIN LEVELS, TISSUE DESTRUCTION AND DISEASE SEVERITY IN INDIVIDUALS WITH SICKLE CELL ANAEMIA
CHAPTER ONE
1.0.       Introduction
Sickle cell anaemia (SCA) is a disorder of the Haemoglobin caused by inheritance of a single abnormal haemoglobin (termed sickle haemoglobin- HbS) which results in physical and chemical modifications of the haemoglobin molecule (Damanhouriet al., 2015). Under conditions of low oxygen concentration, the sickle haemoglobin causes the red blood cell to become fragile and prone to rupture. This condition causes the aggregation of haemoglobin (polymerization) and reduces the overall capacity of the haemoglobin to convey oxygen to tissues by impeding free passage of blood in the vessels (vaso-occlusion) to tissues. Increased rupturing of red blood cells leads to anaemia (reduction in number of red blood cells) (Elias et al., 2012; Damanhouriet al., 2015).
Lactate dehydrogenase (LDH), an enzyme of the glycolytic pathway that catalyzes the inter conversion of pyruvate to lactate is found in abundance in all tissues. It consist of five (5) isoenzymes forms classified according to their electrophoretic mobility. The concentration of these isoenzymes varies from one organ to the other, with LDH1 and LDH2 found primarily in RBCs and heart muscle (Ballas, 2013). There is increased level of LDH in the general blood circulation of individuals with SCA. This is due to the increased breakdown of diseased RBCs and other tissues and has been used as a marker of hemolysis and tissue damage. The haemolysis/tissue damage increases further in individuals with Sickle cell anaemia in crises state (Kato et al., 2006; Ballas 2013). Numerous studies have associated SCA individuals with high LDH level to high prevalence of pulmonary hypertension, renal failure and early death (Kato et al., 2013). High LDH observed in this individuals is as a result of haemolysis and tissue destruction (Mehdiet al., 2013; Alzahriet al., 2015). Despite the importance of LDH as a gold standard marker for tissue damage information about LDH in population suffering from SCA in Nigeria is scarce.
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ASSESSMENT OF THE RELATIONSHIP BETWEEN FOETAL HAEMOGLOBIN LEVELS, TISSUE DESTRUCTION AND DISEASE SEVERITY IN INDIVIDUALS WITH SICKLE CELL ANAEMIA
