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Published on: March 14, 2017

Prevention and management of strokes in patients with sickle cell disease

Miguel R Abboud1, George F Atweh

  • 1Children's Cancer Center of Lebanon, American University of Beirut Medical Center, P.O. Box 11-0236 Riad El-Solh, Beirut 1107-2020, Lebanon. abboudm@aub.edu.lb

Current Hematology Reports
|March 16, 2006
PubMed

Insights

Children with sickle cell disease face high stroke risks. Transfusions can prevent strokes, but better risk stratification and alternative treatments are needed for effective stroke prevention.

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Medical Imaging

Background:

  • Sickle cell disease (SCD) significantly increases stroke risk in children, with overt strokes affecting approximately 11% and silent infarcts being even more common.
  • Current management primarily relies on chronic transfusions to prevent recurrent strokes, a strategy with limitations.

Purpose of the Study:

  • To review current stroke prevention strategies in pediatric sickle cell disease.
  • To highlight the need for improved stroke risk stratification beyond current methods.
  • To explore alternative and adjunctive therapies to chronic transfusions.

Main Methods:

  • Review of the Stroke Prevention Trial in Sickle Cell Anemia (STOP) findings.
  • Discussion of transcranial Doppler (TCD) ultrasonography utility in identifying at-risk children.
  • Exploration of emerging imaging, genetic, and neuropsychological assessment tools for risk stratification.

Main Results:

  • Transcranial Doppler (TCD) ultrasonography effectively identifies children at high risk for stroke, with chronic transfusions reducing first stroke risk by 90% in these patients.
  • A significant proportion (up to 60%) of children with abnormal TCD findings may not develop a stroke, indicating limitations in current risk prediction.
  • Chronic transfusion regimens, while effective, present challenges and are not universally applicable.

Conclusions:

  • More precise stroke risk stratification in pediatric sickle cell disease is crucial, utilizing advanced imaging, genetic, and neuropsychological assessments.
  • Development of alternatives to chronic transfusions, such as hydroxyurea and other pharmacologic agents, is essential for improving outcomes in high-risk patients.

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