Association of Fetal Hemoglobin and Auto-splenectomy in Saudi Adults with Sickle Cell Disease: A Retrospective Study

Abstract: INTRODUCTION: Sickle cell disease (SCD) is the most common hereditary hemoglobinopathy in Saudi Arabia, causing serious complications such as splenomegaly and auto-splenectomy. Radiographic imaging has been found to be a noninvasive tool that can be used to evaluate the spleen changes in patients with SCD. This study evaluates the prevalence of auto-splenectomy in patients with SCD, based on radiographic changes. MATERIALS AND METHODS: Retrospective study in the Eastern Province of Saudi Arabia using data from n = 138 patients with SCD from 2016 to July 2023. The splenic and parenchymal sizes were assessed using either abdominal sonographic or computed tomography studies. Imaging results were correlated with clinical and laboratory data (fetal hemoglobin [HbF] and HbS levels). RESULTS: n = 132 (95.70%) had the HbSS genotype of SCD and n = 6 (4.30%) had HbSβ. The majority of the study sample was found to use hydroxyurea ( n = 91 [65.9%]). One hundred and three (74.6%) had an abnormal spleen size. Auto splenectomy occurred in n = 35 (25.4%), splenomegaly in n = 30 (21.7%), and small spleen in n = 38 (27.5%). HbF level (≥2) was significantly higher among patients taking hydroxyurea ( P = 0.037) and patients with a small spleen ( P = 0.016). CONCLUSIONS: This study showed a positive effect of hydroxyurea treatment on HbF levels and spleen size. However, further research is recommended.