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Updated: Jul 8, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
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.
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.
Abstract:
Sickle cell disease (SCD) is a genetic blood disorder that affects hemoglobin and increases stroke risk, particularly in childhood. This review examines the pathophysiological association between SCD and stroke, the classification of stroke types, risk factors, diagnosis, management, prevention, and prognosis. A comprehensive literature search was conducted via PubMed, Scopus, and Cochrane databases. Relevant studies on SCD and stroke pathophysiology, classification, epidemiology, diagnosis, treatment, and prevention were identified. Sickle cell disease causes red blood cells to become rigid and sickle-shaped, obstructing blood vessels. Recurrent sickling alters cerebral blood flow and damages vessel walls, often leading to ischemic or hemorrhagic strokes (HS). These occur most frequently in childhood, with ischemic strokes (IS) being more common. Key risk factors include a prior transient ischemic attack (TIA), low hemoglobin, and a high leukocyte count. Neuroimaging is essential for diagnosis and determining stroke type. Primary prevention centers on blood transfusions and hydroxyurea for those at high risk. Acute treatment involves promptly restoring blood flow and managing complications. However, significant knowledge gaps remain regarding stroke mechanisms, optimizing screening protocols, and improving long-term outcomes. This review synthesizes current evidence on SCD and stroke to highlight opportunities for further research and standardizing care protocols across institutions. Ultimately, a holistic perspective is critical for mitigating the high risk of debilitating strokes in this vulnerable patient population.
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