Abstract

Background

Red Blood Cell Exchange (RBCX) is a common treatment for pediatric sickle cell disease (SCD). Since inflammation with elevated proinflammatory cytokines plays a crucial role in SCD, this study hypothesized that RBCX might lower these cytokines and aimed to assess the impact of this technique on these markers.

Methods

Prospective and observational study of pediatric SCD patients (HbSS genotype) enrolled in a chronic RBCX program at a Portuguese Hospital from October 2022 to August 2024. Blood samples were collected before and after RBCX to assess hematological and inflammatory markers. Data were analyzed using (Significance level < 0.05); Informed consents were obtained.

Results

Thirty-one children (median age 10 years) were studied: 14 were treatment-naïve, and 17 were previously in a chronic RBCX program. The primary indication for starting the program was cerebrovascular disease prevention (81%). Analysis of 286 RBCXs showed no major adverse events or disease-related hospitalizations. Hemoglobin levels increased by 1.5g/dL post-RBCX; HbS, leukocytes, IL-1, and CRP decreased by 69%, 20%, 21%, and 13%, respectively. Other markers showed no significant changes. IL-1, ferritin, and procalcitonin showed high levels before RBCX; IL-6 showed high levels post-RBCX. Considering only naïve patients, they had higher pre-RBCX IL-1 levels than those with prior RBCX (difference of 22.6 pg/mL); IL-6 increased by 17.3% and IL-1 decreased by 23.9% post-RBCX (p < 0.001).

Conclusions

RBCX safely reduces HbS, leukocytes, and IL-1 levels, suggesting a modulatory effect on inflammation in SCD patients. Further research is needed to explore cytokine mechanisms in SCD.

MeSH

Cytokines, Cells, Anemia, Sickle Cell, Leukocytes, Therapeutics