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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle

Monica L Hulbert1, Robert C McKinstry, JoAnne L Lacey

  • 1Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA.

Blood
|October 14, 2010
PubMed

Insights

Children with sickle cell disease (SCD) and strokes often experience new infarcts despite blood transfusions. This study found that silent cerebral infarcts are common in these children, highlighting the need for better stroke prevention strategies.

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Vascular Medicine

Background:

  • Children with sickle cell disease (SCD) and prior strokes receive transfusions for secondary stroke prevention.
  • Despite transfusions, about 20% experience a second overt stroke.
  • Silent cerebral infarcts are clinically significant in children with SCD.

Purpose of the Study:

  • To test the hypothesis that silent cerebral infarcts occur in children with SCD receiving transfusions for secondary stroke prevention.
  • To investigate the incidence of new cerebral infarcts (overt or silent) in this high-risk pediatric population.

Main Methods:

  • Prospective cohort study of 40 children with SCD and prior strokes at 7 academic centers.
  • Regularly scheduled brain MRI and MRA approximately every 1-2 years.
  • Studies reviewed by a panel of neuroradiologists; eligibility required regular blood transfusion therapy.

Main Results:

  • Progressive cerebral infarcts occurred in 45% (18 of 40) of children during chronic transfusion therapy.
  • Seven children had second overt strokes, and 11 had new silent cerebral infarcts.
  • Worsening cerebral vasculopathy was significantly associated with new cerebral infarction (RR = 12.7, P = .001).

Conclusions:

  • Children with SCD and strokes receiving regular blood transfusions experience silent cerebral infarcts at a higher rate than previously recognized.
  • New cerebral infarcts, both overt and silent, are common despite current secondary stroke prevention strategies.
  • Additional therapeutic strategies are urgently needed for secondary stroke prevention in children with SCD.

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