Effectiveness of a top up transfusion programme in preventing cerebrovascular damage in a birth cohort of sickle cell disease
sunset in florence


sickle cell disease
iron overload

How to Cite

Ibrahim, N., Mahmood, S., O’Driscoll, S., & Chakravorty, S. (2020). Effectiveness of a top up transfusion programme in preventing cerebrovascular damage in a birth cohort of sickle cell disease: Single-centre 10-year Retrospective Analysis . The Physician, 6(2), 1-8. https://doi.org/10.38192/


Regular transfusions are effective in managing strokes in paediatric sickle cell patients. However, there are associated risks, including alloimmunisation and iron overload. This study evaluated the efficacy of top-up transfusions in primary and secondary stroke prevention in a single tertiary paediatric centre in Central London.

Forty-seven children with sickle cell disease who received transfusions in the last decade were included. No patient on a primary stroke prevention transfusion programme had a cerebrovascular event during the study period but 9.5% on secondary stroke prevention programme did. Twenty-one per cent of patients in this cohort converted to exchange transfusions following transfer to adult services, of which 11% had subsequent strokes. Targeted pre-transfusion haemoglobin S % was not always met; 43% of HbS% readings in a 12- month period were above the set target of 30% and 37% were above the set target of 50%. About a third of patients had evidence of severe hepatic iron overload, but no significant cardiac iron. 25% of patients became alloimmunised, but not severe enough to warrant discontinuation of the transfusion programme.

Although transfusions are effective for primary stroke prevention, iron overload remains a significant burden.




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