Initial assessment of the beneficial effect of partial splenectomy in hereditary spherocytosis

摘要: Clinical manifestations of hereditary spherocytosis (HS), the most common red blood cell (RBC) membrane disorder, can be abrogated or markedly reduced by splenectomy. However, concerns regarding risks from overwhelming infections after splenectomy have restricted its use, especially in children. This study was designed to determine if partial decrease hemolytic rate while maintaining phagocytic function spleen. Partial performed 11 children (age 2 13) with HS. The effect on assessed comparing presurgical and postsurgical values for hemoglobin, reticulocyte number, RBC life span. residual splenic using technetium 99m scans enumerating percentage pitted RBCs circulation. There were no complications surgical procedure any individuals. Following splenectomy, hemoglobin increased average 3 g/dL, count decreased 300 x 10(6)/L, span substantially prolonged. Normal uptake noted remnant normal range. is effective decreasing spleen We conclude that use this as treatment disorders warrants consideration, infants under 5 years age who need frequent transfusions.