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.

PubMed

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.