The role of partial splenectomy in children with thalassemia.

Partial splenectomy was performed on 12 patients with thalassemia (9 beta-thalassemia major and 3 Hb H disease) to reduce blood transfusion requirements. The indication for partial splenectomy was the presence of splenomegaly and increased blood transfusion requirements (i.e. Hb drop > 0.5 g per week). Their ages ranged from 3 to 10 years (mean 6.9 years). On follow-up, ranging from 1.1-5.5 years (mean 2.6 years), two of the three patients with Hb H disease required no more blood transfusions while the third continued to receive blood transfusions, but at a lower frequency. For those with beta-thalassemia major, the transfusion requirements and Hb drop per week decreased in the majority of patients. This is specially so during the first 1-2 years following partial splenectomy. In all, about 1/3 of the size of the normal spleen was preserved (either upper or lower pole) which was judged functional as there has been no significant infection in any of the patients, no change in IgM level, no Howell-Jolly bodies and visualization on scintigraphy. Partial splenectomy is recommended to start with for those with Hb H disease. For patients with beta-thalassemia major, partial splenectomy is beneficial as a temporary measure and in those children who are less than 5 years of age, as they are at greater risk of post splenectomy sepsis.