Splenectomy for children with thalassemia.
Twenty-three children with thalassemia (18 with beta-thalassemia major, 3 with Hb H disease, and 2 with thalassemia intermediate) had total splenectomy (nine beta-thalassemia major patients and two thalassemia intermediate patients) or partial splenectomy (nine beta-thalassemia patients and three Hb H disease patients) as part of their management at our hospital. There were 10 males and 1 female in the total splenectomy group (mean age, 7.8 years; range, 4.5-12 years), and 4 males and 8 females in the partial splenectomy group (mean age, 6.9 years; range, 3-10 years). In all, the indication for splenectomy was hypersplenism. In the partial splenectomy group, two children with Hb H disease required no further blood transfusions. The transfusion requirements of the third patient with Hb H disease decreased from 15 to 11 transfusions per year (from 1.2 g/week Hb drop preoperatively to 0.7 g/week postoperatively), but subsequently his transfusion requirements increased as a result of an increase in the size of splenic remnant. He underwent total splenectomy 1.5 years post-partial splenectomy. For those with beta-thalassemia major who had partial splenectomy, there was a reduction in the number of blood transfusions from a preoperative mean of 15.2 transfusions per year (range, 11-22 transfusions per year) to a postoperative mean of 8.2 transfusions per year (range, 2-11 transfusions per year). Their Hb drop decreased from a preoperative mean of 1.6 g/week (range, 0.8-3.5 g/week) to a postoperative mean of 0.5 g/week (range, 0.2-0.75 g/week). Subsequently and as a result of increase in the size of splenic remnant, their transfusion requirements increased, but none of them to this point have required total splenectomy. Eleven children had total splenectomy. Their postsplenectomy transfusion requirements decreased from a preoperative mean of 17.8 transfusions per year (range, 12-23 transfusions per year) to a postoperative mean of 10 transfusions per year (range, 8-12 transfusions per year), and their Hb drop decreased from a preoperative mean of 1.8 g/week (range, 0.5-2.3 g/week) to a postoperative mean of 0.45 g/week (range, 0.3-0.65 g/week). In conclusion, total splenectomy is beneficial for children with thalassemia and hypersplenism because it reduces their transfusion requirements. Partial splenectomy may be beneficial for those with Hb H disease. However, for those with beta-thalassemia, partial splenectomy is beneficial in reducing their transfusion requirements only as a temporary measure, and it is recommended for children who are less than 5 years of age.