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Updated: Aug 27, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
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A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy

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Stroke in sickle cell disease and the promise of recent disease modifying agents

Ava Runge1, Danielle Brazel2, Zahra Pakbaz3

  • 1University of California Irvine School of Medicine, CA, USA.

Journal of the Neurological Sciences
|September 23, 2022
PubMed

Insights

Sickle cell disease (SCD) patients face high risks of stroke and silent cerebral infarcts. Current prevention options are limited, necessitating research into novel therapies and addressing healthcare disparities.

Area of Science:

  • Neurology
  • Hematology
  • Vascular Medicine

Background:

  • Sickle cell disease (SCD) is a prevalent inherited hemoglobinopathy in the US.
  • Cerebrovascular complications, including stroke and silent cerebral infarcts (SCI), are frequent and severe in SCD patients.
  • Over half of SCD patients experience SCI by age 30, and a significant percentage suffer overt strokes by age 40.

Purpose of the Study:

  • To review the impact of cerebrovascular disease in SCD.
  • To examine the pathophysiology of stroke and cerebral fat embolism in SCD.
  • To assess current and potential future stroke prevention strategies for SCD.

Main Methods:

  • This is a literature review.
  • The review synthesizes existing research on SCD, cerebrovascular disease, and stroke.
  • Pathophysiology and treatment options are discussed based on published data.

Main Results:

  • Stroke and SCI are major complications of SCD.
  • Current stroke prevention in SCD is limited to hydroxyurea and blood transfusions.
  • Novel disease-modifying agents show promise for SCD complications and may aid stroke prevention by targeting hemolysis and vaso-occlusion.
  • The role of aspirin and anticoagulation in SCD stroke requires further study.

Conclusions:

  • Cerebrovascular disease poses a significant burden in SCD.
  • Limited treatment options exist for stroke prevention in SCD.
  • Further research into novel agents and addressing healthcare disparities is crucial for advancing SCD care and stroke prevention.

Keywords:
Cerebral fat embolismEpidemiologyPreventionSickle cellSilent cerebral infarctStrokeTranscranial doppler

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