M. Verla1, G. Airewele2, C. Style1, H. Sriraman1, O. Olutoye1 1Baylor College of Medicine, Michael E. DeBakey Department of Surgery,Division Of Pediatric Surgery, Texas Children’s Hospital,Houston, TX, USA 2Baylor College Of Medicine,Department Of Pediatrics, Section Of Hematology-Oncology, Texas Children’s Hospital,Houston, TX, USA
Introduction: Sickle cell disease (SCD) is typically associated with auto-splenectomy from splenic infarction. Surgical splenectomy is performed on those with sequestration crises or hypersplenism. Hydroxyurea therapy decreases the frequency and severity of sickle cell crises and thus the auto-splenectomy associated with sickle cell disease.. The purpose of this study was to determine if hydroxyurea therapy is associated with 1) an increase in the incidence of surgical splenectomy and 2) a later age at surgical splenectomy.
Methods: We performed a retrospective review of children with SCD who underwent a surgical splenectomy at our children’s hospital between January 1990 and December 2017. Patient demographics, type of SCD, hydroxyurea use, and peri-operative data were collected. Patients were further stratified into two groups, pre-2005 and post-2005, based on the year when hydroxyurea use steadily increased at our institution. Data were analyzed using chi-square analysis and two-way multivariate analysis of variance. A p-value < 0.05 was considered statistically significant.
Results: Over the 27-year period, a total of 2,910 patients with SCD were identified and 125 children had a splenectomy. Of these, 20% (n=21) received hydroxyurea for at least 6 months prior to surgical splenectomy. Splenic sequestration and hypersplenism were the most common indications (96%) for splenectomy at a median age of 5 years (IQR: 2.6 – 9.9). The cumulative incidence of splenectomy was 4.9% pre-2005 versus 3.5% post-2005. Ninety-four children (78%) had HbSS, of whom 18 had hydroxyurea therapy for at least 6 months. Those who had a long-term history of hydroxyurea therapy had a splenectomy at a median age of 6 years (IQR: 3.4–8.9) versus 3 years (IQR: 2.2–6.4) for those who did not have a long-term history of hydroxyurea use (p=0.03, Figure 1). Regardless of the pre- or post-2005 stratification, all HbSS patients on hydroxyurea therapy had their splenectomy at a later age.
Conclusion: Although the incidence of surgical splenectomy does not appear to have increased with the introduction of hydroxyurea therapy, patients receiving hydroxyurea long-term are undergoing surgical splenectomy at an older age.
