Overview of the Association Between the Pathophysiology, Types, and Management of Sickle Cell Disease and Stroke

Faisal Hakami1, Essam Alhazmi1, Wafa M Busayli1

  • 1Medicine, Faculty of Medicine, Jazan University, Jazan, SAU.

Cureus
|December 18, 2023
PubMed

Insights

Sickle cell disease (SCD) significantly elevates stroke risk in children due to red blood cell sickling. Early detection and prevention strategies like transfusions are crucial for managing this serious complication.

Area of Science:

  • Hematology
  • Neurology
  • Pediatrics

Background:

  • Sickle cell disease (SCD) is a genetic disorder impacting hemoglobin, leading to rigid, sickle-shaped red blood cells.
  • These abnormal cells obstruct blood flow, increasing the risk of ischemic or hemorrhagic strokes, particularly in childhood.

Purpose of the Study:

  • To review the pathophysiology, classification, risk factors, diagnosis, management, and prevention of stroke in sickle cell disease.
  • To identify knowledge gaps and opportunities for research and standardized care protocols.

Main Methods:

  • Comprehensive literature search of PubMed, Scopus, and Cochrane databases.
  • Identification and synthesis of relevant studies on SCD and stroke pathophysiology, epidemiology, diagnosis, and treatment.

Main Results:

  • SCD causes vessel obstruction and damage, leading to frequent childhood strokes, predominantly ischemic.
  • Key risk factors include prior transient ischemic attack (TIA), low hemoglobin, and high leukocyte count.
  • Neuroimaging is vital for diagnosis; primary prevention involves transfusions and hydroxyurea.

Conclusions:

  • Despite advances, significant knowledge gaps persist in understanding stroke mechanisms and optimizing long-term outcomes in SCD patients.
  • A holistic approach is essential to mitigate stroke risk and improve care standardization for this vulnerable population.

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