Developing treatment for sickle cell disease

Abstract

Sickle cell disease pathophysiology results from sickle haemoglobin polymerisation and its effects on the sickle erythrocyte and the vasculature. Many of the abnormalities of sickle cell disease are secondary to the damage caused by the polymer and the injured red cell. Pharmacological treatment of the disease is focused on the inhibition of sickle haemoglobin polymerisation, prevention or repair of red cell dehydration and interruption of the interaction of sickle cells with the endothelium.

• butyrate
• cation transport
• cell membrane
• endothelium
• fetal haemoglobin
• histone deacetylase
• hydroxyurea
• nitric oxide