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Updated: Jun 6, 2026
Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Effect of chronic red cell transfusion therapy on vasculopathies and silent infarcts in patients with sickle cell
Elsie Gyang1, Kristen Yeom, Carolyn Hoppe
1Department of Pediatrics, Stanford University School of Medicine, Palo Alto, California, USA.
Insights
Chronic red cell transfusions (CTX) may not fully prevent silent infarcts or halt cerebral vasculopathy in sickle cell disease (SCD) patients, particularly those with prior strokes.
Area of Science:
- Neurology
- Hematology
- Vascular Medicine
Background:
- Chronic red cell transfusions (CTX) are standard for stroke prevention in sickle cell disease (SCD).
- The effect of CTX on silent infarcts (SI) and cerebral vasculopathy is not well understood due to limited serial brain imaging.
- Sickle cell disease (SCD) patients face significant risks of stroke and cerebrovascular complications.
Purpose of the Study:
- To evaluate the impact of CTX on silent infarcts (SI) and cerebral vasculopathy in SCD patients.
- To assess whether CTX prevents new SIs or progression of existing vasculopathy.
- To compare outcomes between primary and secondary stroke prophylaxis in SCD patients on CTX.
Main Methods:
- Retrospective review of MRI reports from SCD patients receiving CTX for stroke prophylaxis.
- Analysis of new silent infarcts (SI) and cerebral artery vasculopathy progression.
- Comparison of outcomes based on CTX indication (primary vs. secondary prophylaxis).
Main Results:
- New silent infarcts (SI) occurred in 17.6% of patients (rate of 5.42 per 100 patient-years).
- Cerebral vasculopathy was present in 65% and progressed in 63% of affected patients.
- Patients on CTX for secondary prophylaxis showed more SIs and progressive vasculopathy than those on primary prophylaxis.
Conclusions:
- CTX adherence does not guarantee prevention of silent infarcts (SI) or halt cerebral vasculopathy progression in SCD.
- Patients with a history of overt stroke are at higher risk for continued cerebrovascular complications despite CTX.
- Further research is needed to optimize stroke prevention strategies in sickle cell disease (SCD).
Abstract:
Regular, chronic red cell transfusions (CTX) have been shown to be effective prophylaxis against stroke in sickle cell disease (SCD) in those at risk. Because serial brain imaging is not routinely performed, little is known about the impact of CTX on silent infarcts (SI) and cerebral vascular pathology. Thus, we retrospectively evaluated the magnetic resonance imaging reports of a cohort of SCD patients who were prescribed CTX for either primary or secondary stroke prophylaxis. Seventeen patients with Hb SS were included (mean age 15 years, mean follow-up 4.3 years). Eight patients were on CTX for primary prophylaxis. New SI occurred in 17.6% of patients corresponding to an SI rate of 5.42 per 100 patient-years. Vasculopathy of the cerebral arteries was present in 65% of patients and progressed in 63% of these patients. Those who developed progressive vasculopathy were on CTX for an average of 8 years before lesions progressed. Patients on CTX for secondary prophylaxis had more SIs and evidence of progressive vascular disease than patients on CTX for primary prophylaxis. We conclude that adherence to CTX does not necessarily prevent SI or halt cerebral vasculopathy progression, especially in those with a history of overt stroke.
