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G366(P) Systematic review on the use of hydroxycarbamide in reducing transcranial doppler velocities in children with sickle cell anaemia and whether this results in stroke prevention

Introduction Sickle cell anaemia is a common inherited haemoglobinopathy in which distorted red blood cells cause vaso-occlusion and ischaemic-reperfusion injury in various organs. Strokes are a severe complication of sickle cell anaemia in children and results in significant morbidity. The mainstay treatment for stroke prevention is chronic blood transfusions, however this is not without its risks and complications. By measuring transcranial Doppler velocity, at-risk patients can be identified and preventative treatment initiated. Hydroxycarbamide has been shown to reduce transcranial doppler velocities in sickle cell patients and therefore Hydroxycarbamide has been considered a potential alternative candidate to blood transfusions with less adverse effects. Aim To assess whether hydroxycarbamide was effective in reducing transcranial doppler velocities and if this translated into efficient stroke prevention in children with sickle cell anaemia. Search criteria The Cochrane database, PUBMED and Google scholar were searched. Prospective, Randomised Control and Observational trials were included. Results Searches identified 6 eligible studies (n=365). All studies unanimously showed an initial reduction in TCD velocities. One trial was able to show non-inferiority(p=8.82*10E-16) of hydroxycarbamide to chronic blood transfusions. Nevertheless, another study identified a reversion to abnormal TCD velocities after a mean follow up period of 1.1 years. Conclusions In conclusion, Hydroxyurea appears to be useful in reducing TCD velocities in both abnormal (defined as TCD velocity >200 cm/s) and conditional (>180 cm/s) TCD velocities. In terms of stroke prevention, there is no significant decrease in occurrence or at least non-superiority. There is no consensus on the role of hydroxycarbamide as a prophylaxis of stroke.