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Published on: March 14, 2017
The prevention and management of stroke in sickle cell anaemia
N Qureshi1, B Lubin, M C Walters
1Oakland and Children's Hospital Oakland Research Institute, Children's Hospital & Research Center, Oakland, CA, USA.
Insights
Stroke is a major complication of sickle cell anaemia in children. Regular red blood cell transfusions are standard for preventing and treating stroke, with hematopoietic cell transplantation as a potential cure.
Area of Science:
- Hematology
- Pediatric Neurology
- Vascular Medicine
Background:
- Stroke is a significant clinical complication of sickle cell anaemia, affecting 5-10% of affected children.
- Central nervous system (CNS) damage necessitates interventions to prevent recurrent or progressive neurological deficits.
Purpose of the Study:
- To review standard and novel red blood cell (RBC) transfusion techniques for stroke prevention and treatment in children with sickle cell anaemia.
- To discuss alternative therapies and hematopoietic cell transplantation as a curative option for sickle cell anaemia-related stroke.
Main Methods:
- Literature review of standard and novel RBC transfusion techniques.
- Analysis of alternative therapies and hematopoietic cell transplantation for sickle cell anaemia stroke.
- Consideration of transcranial Doppler screening for high-risk children.
Main Results:
- Regular RBC transfusions are the standard of care for preventing recurrent or progressive CNS damage in children with sickle cell anaemia.
- Transcranial Doppler screening identifies children at high risk for stroke who benefit from prophylactic RBC transfusions.
- Hematopoietic cell transplantation is the only curative treatment for sickle cell anaemia.
Conclusions:
- RBC transfusions are crucial for managing stroke risk in pediatric sickle cell anaemia.
- Alternative therapies and hematopoietic cell transplantation warrant further investigation for comprehensive stroke management.
- Future applications of hematopoietic cell transplantation in this clinical setting require continued evaluation.
Abstract:
Perhaps the most important clinical complication of sickle cell anaemia is stroke, an event that occurs in approximately 5-10% of children who inherit this disorder. To prevent recurrent or progressive CNS damage, the institution of regular red blood cell (RBC) transfusions is the standard of care. In addition, children at high risk of developing stroke, as screened by transcranial Doppler, also benefit from regular RBC transfusions for stroke prevention. In this review, standard and novel techniques of RBC transfusion, and also alternative therapies to treat children with or at risk for stroke are considered. In addition, haematopoietic cell transplantation, the only curative option for sickle cell anaemia, is considered, and speculation about its present and future application in this clinical setting is discussed.
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