The appropriate selection of blood for transfusion in the ongoing treatment of patients with sickle cell disease is of vital importance. Here, Hannah Caldwell examines the role of sickle solubility testing and molecular genotyping in the care of SCD patients in Northern Ireland.
Sickle cell disease (SCD) is a group of haemoglobinopathies that contain mutations in the gene encoding the beta subunit of haemoglobin. It is characterised by the presence of sickle haemoglobin. The sickle cell mutation occurs when glutamine is replaced by valine at the sixth position of the β-globin chain resulting in sickle-shaped red blood cells, progressive multi-organ failure and an increase in mortality.1,2
Complications of SCD
Northern Ireland population
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