Related Experiment Video
Updated: Jul 19, 2026

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Treatment and prevention of stroke in children with sickle cell disease
Shyamal H Mehta1, Robert J Adams
1Department of Neurology, Medical College of Georgia, 1120 15th Street, Augusta, GA 30912, USA. rjadams@mcg.edu.
Insights
Regular blood transfusions significantly reduce stroke risk in children with sickle cell disease (SCD). Transcranial Doppler (TCD) screening identifies high-risk children, making transfusions a proven stroke prevention strategy.
Area of Science:
- Neurology
- Hematology
- Pediatrics
Background:
- Stroke is a significant complication in children with sickle cell disease (SCD), ranging from silent infarcts to severe, disabling lesions.
- Intracranial hemorrhages in SCD can result from aneurysms, moyamoya disease, or venous issues.
- While acute stroke treatments are limited, evidence supports stroke prevention strategies in this population.
Purpose of the Study:
- To review the evidence for stroke prevention in children with sickle cell disease.
- To highlight the effectiveness of transcranial Doppler (TCD) screening and blood transfusions in primary stroke prevention.
- To discuss current and potential secondary stroke prevention methods.
Main Methods:
- The Stroke Prevention Trial in Sickle Cell Anemia (STOP) randomized high-risk children (TCD velocity ≥ 200 cm/s) to transfusion or no transfusion.
- STOP2 evaluated the impact of discontinuing transfusions on stroke risk.
- Review of existing literature on stroke prevention in SCD.
Main Results:
- The STOP trial demonstrated a 90% reduction in first-time stroke among children receiving regular blood transfusions.
- Discontinuing transfusions in STOP2 led to a high rate of abnormal TCD values and recurrent strokes.
- TCD screening and chronic transfusion (maintaining HbS < 30%) is the only established primary stroke prevention strategy for SCD.
Conclusions:
- Transcranial Doppler (TCD) screening and maintaining low hemoglobin S levels through chronic transfusion is the sole proven method for primary stroke prevention in children with SCD.
- Further research is needed for secondary stroke prevention, with hydroxyurea under investigation.
- Bone marrow transplantation shows potential for stroke prevention but requires more data.
Abstract:
Stroke is one of the major complications in children with sickle cell disease (SCD). Ischemic stroke is associated with small asymptomatic subcortical infarcts to large territorial lesions causing major disability. Intracranial hemorrhages may be caused by aneurysm rupture or by leakage from moyamoya vessels or venous sources. There have been no acute stroke treatment studies in SCD, but hydration and exchange transfusion are often recommended. However, there is an evidence base for primary and to some extent secondary stroke prevention. Primary prevention of stroke was demonstrated in the Stroke Prevention Trial in Sickle Cell Anemia (STOP), in which children with transcranial Doppler (TCD) mean blood flow velocities of 200 cm/second (previously shown to indicate high stroke risk) or higher were randomized to either regular blood transfusions or no transfusion. The study showed a very significant 90% reduction in first stroke with transfusion. In STOP2, discontinuing transfusions after 30 months or more (even with normal TCD) resulted in a high rate of reversion to abnormal TCD values and stroke. TCD screening of all children with SCD, and initiation and maintenance of chronic transfusion to maintain hemoglobin S below 30% in the high-risk group, is the only proven prevention strategy for stroke in SCD. Hydroxyurea is being studied as secondary stroke prevention at this time. No recommendation specific to SCD regarding the use of antiplatelet agents or anticoagulants in ischemic stroke can be made. Bone marrow transplantation can be curative for SCD, and limited data support its use to prevent stroke in SCD.
Related Concept Videos
Venous Thrombosis III: Interprofessional Care
Ischemic Stroke ll: Pathophysiology
Hemorrhagic Stroke ll: Pathophysiology
Hemorrhagic Stroke l: Introduction
Ischemic Stroke l: Introduction
Atherosclerosis IV: Nursing Management
