Splenectomy in Children with Beta Thalassemia Major and Sickle Cell Disease: A 4-Year Experience from Iraq

Abstract Background: Splenectomy is indicated as a therapeutic option in different types of hereditary blood disorders. Objective: The aim of this study is to determine the outcome of splenectomy in children with beta-thalassemia major (BTM) and sickle cell disease (SCD). Materials and Methods: A retrospective study was conducted in Basrah-Iraq on children with BTM and SCD who had undergone open splenectomy in the period between January 2016 and December 2019 at our institution. Results: The study included 87 children (49 males and 38 females). 59.8% with SCD and 35 (40.2%) with BTM. The mean age was 8.46 range (3–15) years. The mean weight was 23.51 range (11–45) kg. In SCD, the major indication (71.1%) was acute splenic sequestration (ASS). Postsplenectomy outcome in BTM included improvement in the median hemoglobin (Hb) level by 2.1 g/dL, reduction in the median packed red blood cells (PRBC) transfusion frequency by 19/year, and reduction in the median PRBC requirement by 190 mL/kg/year. In SCD, improvement in the median Hb level by 1.55 g/dL, reduction in the median PRBC transfusion frequency by 4.5/year, and reduction in the median PRBC requirement by 45 mL/kg/year. In addition, splenectomy eliminated life-threatening ASS crisis. Postoperative complications were observed in 6.9% of patients. Conclusions: SCD was the main hematological condition requiring splenectomy. ASS was the main indication for splenectomy in SCD. Splenectomy has proved beneficial in the management of SCD and BTM patients. Furthermore, it is now recognized as a safe surgery with a low complication rate.