The sickle cell disorders (SCD) comprise homozygous sickle cell anaemia (SS), SS with~ t-
thalassaemia, sickle cell/haemoglobin C disease (SC), sickle cell/fl-thalassaemia, and other
less common variants. These inherited disorders of haemoglobin (Hb) structure are
characterized by chronic haemolysis, frequent infections, and recurring episodes of
obstruction of the microvasculature by poorly deformable erythrocytes. Vascular obstruction
causes acute and chronic tissue ischaemia and, ultimately, results in organ damage through …