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Published on: November 5, 2019
Pathophysiology of stroke in sickle cell disease
Cheryl A Hillery1, Julie A Panepinto
1Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin 53201-2178, USA. chillery@bcsew.edu
Insights
Stroke in sickle cell disease (SCD) impacts motor and cognitive abilities. Pathophysiology involves complex vascular issues, including red cell adhesion and inflammation, contributing to both symptomatic and silent strokes.
Area of Science:
- Neurology
- Hematology
- Vascular Biology
Background:
- Stroke is a significant complication in sickle cell disease (SCD), affecting both motor and cognitive functions.
- Symptomatic strokes in SCD are linked to large cerebral artery intimal disease.
- Silent strokes, often caused by microinfarcts, suggest underlying microvascular disease in SCD patients.
Purpose of the Study:
- To elucidate the poorly understood pathophysiology of stroke in sickle cell disease.
- To explore the potential mechanisms contributing to cerebral vasopathology in SCD, considering various vascular injury sites.
Main Methods:
- Review of existing literature on stroke natural history and pathophysiology in SCD.
- Analysis of proposed contributing factors to cerebral vasopathology, including cellular and molecular mechanisms.
Main Results:
- Stroke in SCD affects both motor and cognitive functions.
- Pathophysiology is complex and likely varies with the site of vascular injury.
- Potential contributors include red cell adhesion, oxidative stress, inflammation, and coagulation system activation.
Conclusions:
- The pathophysiology of stroke in sickle cell disease is multifactorial and not fully understood.
- Understanding these mechanisms is crucial for developing targeted therapies.
- Cerebral vasopathology in SCD may involve a combination of factors affecting large and small cerebral vessels.
Abstract:
Stroke affects both motor and cognitive function in patients with sickle cell disease (SCD). Symptomatic stroke is associated with intimal disease of the large cerebral arteries. Silent stroke, defined as cerebral infarction in the absence of overt clinical neurologic symptoms, is often due to microinfarcts suggestive of microvascular disease. While the natural history of stroke in SCD is well described, the pathophysiology remains poorly understood and probably varies with the site of vascular injury. Increased red cell adhesion, oxidative injury of the vessel wall, inflammation, abnormal vasomotor tone regulation, and increased activity of the coagulation system all may contribute to cerebral vasopathology in SCD.
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