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Updated: Aug 2, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
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Managing the Cerebrovascular Complications of Sickle Cell Disease: Current Perspectives

Jennifer Light1, Maria Boucher1, Jacquelyn Baskin-Miller1

  • 1Pediatric Hematology-Oncology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.

Journal of Blood Medicine
|April 21, 2023
PubMed

Insights

Protecting brain function in sickle cell disease (SCD) is crucial due to stroke risks. Screening and management, including transfusions and hydroxyurea, can prevent serious cerebrovascular complications.

Area of Science:

  • Neurology
  • Hematology
  • Pediatrics

Background:

  • Sickle cell disease (SCD) poses significant threats to neurocognitive function and life due to cerebrovascular complications.
  • Unmanaged SCD leads to strokes in 11% of children and 24% of adults.
  • Silent cerebral infarction (SCI) affects over 39% of children and 50% of adults with SCD.

Purpose of the Study:

  • To highlight the importance of protecting brain function in individuals with SCD.
  • To review current screening, management, and emerging therapies for SCD-related cerebrovascular complications.
  • To emphasize the need for proactive neuroprotection strategies.

Main Methods:

  • Transcranial Doppler ultrasound (TCD) for stroke risk screening in children with SCD.
  • Management strategies including chronic red blood cell (RBC) transfusion, hydroxyurea, and hematopoietic stem cell transplant.
  • Neurocognitive evaluation for silent cerebral infarction (SCI) management.

Main Results:

  • TCD velocities above 200 cm/s indicate high stroke risk, preventable with chronic RBC transfusion.
  • Chronic RBC transfusion requires management of complications like iron overload.
  • Hydroxyurea offers an alternative for stroke prevention in specific populations.
  • Hematopoietic stem cell transplant can prevent cerebrovascular complications.
  • Investigational therapies include voxelotor, crizanlizumab, and gene therapy.

Conclusions:

  • Protecting brain function is paramount for individuals with SCD.
  • Established methods like TCD screening and RBC transfusions are vital for stroke prevention and management.
  • Emerging therapies show promise for treating SCD-related cerebrovascular issues.

Keywords:
moyamoya syndromesilent cerebral infarctionstem cell transplantationstroketransfusion

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