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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Real-world assessment of acute red cell exchange for stroke in sickle cell disease
Samuel R Wilson1,2, Denis Noubouossie3,4, Jane A Little1,2
1Division of Hematology, Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
Insights
Red cell exchange (RCE) therapy for cerebrovascular accidents (CVA) in sickle cell disease (SCD) showed improved outcomes. Higher post-RCE hematocrit was linked to reduced death or persistent neurological symptoms in SCD patients.
Area of Science:
- Neurology
- Hematology
- Pediatrics
Background:
- Cerebrovascular accidents (CVA) are severe complications in sickle cell disease (SCD).
- Chronic transfusion therapy reduces CVA incidence in SCD.
- Acute management's impact on neurological outcomes in SCD-related CVA is understudied.
Purpose of the Study:
- To examine neurological outcomes in children and adults with SCD receiving acute red cell exchange (RCE) for neurological events.
- To identify factors associated with improved outcomes after acute RCE therapy.
Main Methods:
- Retrospective analysis of 29 children and adults with SCD treated with acute RCE for neurological events.
- Data collected on prior CVA history, chronic transfusions, comorbidities (diabetes, hypertension), and thrombolytic therapy use.
- Statistical analysis to determine the association between post-RCE hematocrit and neurological outcomes.
Main Results:
- Higher post-RCE hematocrit was associated with decreased odds of death or persistent neurological symptoms at hospital discharge (OR 0.69, 95% CI: 0.45-0.94, p=0.017).
- 12 (41%) patients had a prior CVA history, and 12 (41%) were on chronic transfusions.
- Only one adult received thrombolytic therapy due to ineligibility in most cases.
Conclusions:
- Acute red cell exchange (RCE) is a primary therapy for neurological events in SCD.
- Higher post-RCE hematocrit may improve neurological outcomes and reduce mortality in SCD patients with CVA.
- Further prospective studies are needed to optimize acute management strategies for SCD-related CVA.
Abstract:
Cerebrovascular accidents (CVA) are one of the most devastating complications in sickle cell disease (SCD). Chronic transfusion therapy has been established for primary and secondary prevention of CVA in SCD, resulting in a notable reduction in CVA incidence. For individuals with SCD presenting with CVA, the impact of acute management on neurological outcomes is not well studied. Herein, we examine the neurological outcomes of 29 children and adults with SCD who received acute red cell exchange (RCE) as primary therapy for neurological events at a single institution. Twelve (41%) individuals had a prior history of CVA, and 12 (41%) were on chronic transfusions. Among 13 adults, 3 (23%) had a history of diabetes and 4 (31%) had a history of hypertension. One adult (7.7%) received thrombolytic therapy; the remainder were ineligible due to age, timing of presentation, intracranial haemorrhage or transient symptoms. Higher post-RCE haematocrit was associated with decreased odds of death or persistent neurological symptoms at hospital discharge (OR 0.69, 95% CI: 0.45-0.94, p = 0.017). Optimal acute management of adults living with SCD presenting with CVA remains an understudied yet important topic. Future prospective studies may determine how best to tailor acute management to improve neurological outcomes.

