A Cochrane review included 2 studies with a total of 209 children. One trial (n=130) compared a chronic transfusion regimen for maintaining sickle haemoglobin lower than 30% with standard care in children with sickle cell disease (SCD) judged as high-risk for first stroke. The trial was terminated early, as 11 children in the standard care group suffered a stroke compared to one in the transfusion group (92% relative risk reduction). The transfusion group had a high complications rate: iron overload; alloimmunisation; and transfusion reactions. The second trial (n=79) investigated risk of stroke when transfusion was stopped after at least 30 months. The trial closed early due to a significant difference in risk of stroke between participants who stopped and those who continued (for whom it was deemed unsafe to recommend discontinuation), as measured by abnormal velocities on Doppler examinations, OR 0.02 (95% CI 0.00 to 0.43).
Comment: The quality of evidence is downgraded by study quality (stopping early for benefit).