Treatment with hydroxyurea might be associated with higher rates of surgical splenectomy in children with sickle cell disease (SCD) as well as younger age at intervention, according to a recent retrospective cross-sectional study published in the Journal of Surgical Research.

To investigate the correlation between the widespread SCD therapy agent and surgical splenectomy among the pediatric population, the study authors searched through the Pediatric Health Information System for all SCD International Classification of Diseases 9/10 diagnosis codes and splenectomy procedure codes from January 1, 2005, to December 31, 2020. Proof of hydroxyurea treatment was derived from hospital admission records in which hydroxyurea was listed as a medication.

In addition, the researchers compared the rates of surgical splenectomy, age at splenectomy, hospital length of stay, and incidence of blood transfusion during splenectomy admission among patients receiving hydroxyurea vs those not receiving the medication.

The search identified 28,520 patients, who were then classified into 4 cohorts for analysis: total population, hemoglobin-SS (Hb-SS) genotype, hemoglobin-SC genotype, and other diseases.

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After analyzing the data, it was noted that patients with SCD receiving hydroxyurea had a significantly higher rate of surgical splenectomy than those who were not treated (7.2% vs 3.2%). Moreover, patients in the hydroxyurea group underwent surgical splenectomy at a much younger age in the total SCD cohort and the Hb-SS subgroup.

No significant variation was found between treatment groups in the length of hospital stay or incidence of blood transfusion during surgical splenectomy admission.

“In the Hb-SS population, the higher splenectomy rate among patients taking hydroxyurea is likely due to several reasons. The first is the preservation of the spleen from autosplenectomy, perhaps through fewer vaso-occlusive crises known to result in tissue loss,” Menchaca and colleagues wrote. “The second reason could be that hydroxyurea is a marker for more severe disease.”

Prior to the popularization of hydroxyurea as a treatment option for SCD, patients would often experience early splenic damage and subsequent tissue atrophy regarded as autosplenectomy. However, a large proportion of people whose spleen remains functional might eventually need surgery due to a splenic sequestration crisis or hypersplenism.


Menchaca AD, Style CC, Villella AD, et al. Pediatric sickle cell disease patients on hydroxyurea have higher rates of surgical splenectomy. J Surg Res. Published online December 2, 2022. doi:10.1016/j.jss.2022.11.026