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December 8, 2025BloodOpen Access

Complement dysregulation during vaso-occlusive episodes can predict development of acute chest syndrome in pediatric patients with sickle cell disease

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Authors

AWAdrianna WestbrookEmory UniversityJYJustin YooEmory UniversityJJJayre JonesEmory University

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Overview

Prospective study shows complement dysregulation and hemolysis predict acute chest syndrome risk in sickle cell disease, highlighting potential therapeutic targets.

Key Points

  • The aim is to evaluate the role of complement dysregulation as a predictor for acute chest syndrome in pediatric sickle cell disease patients during vaso-occlusive episodes.
  • Single institution, prospective study conducted at Children's Healthcare of Atlanta
  • Enrolled sickle cell disease patients aged 0-21 with vaso-occlusive episodes
  • Blood samples collected at admission and follow-up to measure complement levels and biomarkers
  • Statistical analyses included receiver operating characteristic curves and logistic regression
  • Complaints of acute chest syndrome associated with elevated complement levels and hemolysis
  • Significant predictors identified include WBC, C4d, Bb, C3a, and C5a
  • Upper 75th percentile of LDH significantly increased odds for developing acute chest syndrome
  • Hydroxyurea associated with lower odds of acute chest syndrome
  • Developing acute chest syndrome linked to increased need for oxygen support and red cell transfusions

Cite This Study

Westbrook et al. (2025) studied this question.

synapsesocial.com/papers/69362f7d4fa91c937236e493https://doi.org/10.1182/blood-2025-1167
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Investigation of predictive markers of disease complications in children with sickle cell disease: Preliminary findings from a multicenter prospective inception cohort study2025
  2. 2Rapidly progressive acute chest syndrome is associated with complement-mediated cell injury in adults with sickle cell disease2025
  3. 3Predictors of Acute Chest Syndrome Following Vaso-Occlusive Crisis in Pediatric Sickle Cell Disease2026 · 1 citations
  4. 4Deep neutrophil phenotyping uncovers complement-mediated neutrophil dysregulation in sickle cell disease2025
  5. 5Revisiting acute chest syndrome (ACS) associated with sickle cell disease (SCD) vaso-occlusive pain episodes (VOE): Insight from a prospective, multicenter Phase-3 randomized controlled trial2025 · 1 citations