How many children with sickle cell disease (SCD) and stroke experience subsequent events if long-term transfusion therapy is not provided?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Unless long-term transfusion therapy is provided, 70-90% of children who experience a single stroke have subsequent events. DeBaun et al reported that in children with SCD, regular blood transfusions significantly reduced the rate of cerebral infarct recurrence. [97]

As patients grow into adulthood, the transfusion frequency may be decreased, but whether it can be discontinued remains unclear. Many believe that lifelong transfusion therapy is necessary to completely eliminate recurrences in patients with SCD. The AHA/ASA primary stroke prevention guidelines endorse (pending further study) the continued use of transfusion, even in those with TCD velocities that return to normal. [45] Iron overload from repeated transfusions requires chelation therapy after 2-3 years.


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