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Results of the splenectomy in children with thalassemia
Insights
Splenectomy significantly improved hematocrit and reduced transfusion needs in children with beta-thalassemia/E, hemoglobin H disease, and beta-thalassemia major. Growth and liver size showed varied responses post-surgery.
Area of Science:
- Pediatric Hematology
- Surgical Outcomes
- Genetic Blood Disorders
Background:
- Beta-thalassemia and hemoglobin H disease are inherited blood disorders requiring significant medical management.
- Splenectomy is a potential intervention for these conditions, but its long-term effects require thorough evaluation.
Purpose of the Study:
- To retrospectively analyze the outcomes of splenectomy in children with beta-thalassemia/E, hemoglobin H disease, and beta-thalassemia major.
- To assess the impact of splenectomy on hematological parameters, transfusion requirements, growth, and postoperative complications.
Main Methods:
- Retrospective study of 69 children followed for at least 2 years pre- and post-splenectomy.
- Analysis of hematocrit levels, annual blood transfusion frequency, liver size, growth velocity (height and weight), and postoperative complications.
Main Results:
- Significant increases in hematocrit observed across all studied conditions.
- Marked reduction in annual blood transfusion requirements, with none needed for hemoglobin H disease post-splenectomy.
- Varied changes in liver size and growth velocity depending on the specific thalassemia type; some postoperative complications occurred, including pneumonia, septicemia, and wound bleeding.
Conclusions:
- Splenectomy offers significant hematological benefits for children with various thalassemia syndromes.
- Careful monitoring for postoperative complications and long-term effects on growth and organ size is essential.
Abstract:
Sixty-nine children with beta-thalassemia/E disease, hemoglobin H disease and beta-thalassemia major who were followed for at least 2 years before and after splenectomy were studied retrospectively for the results of the splenectomy. It was found that. 1. The hematocrit increased significantly in beta-thalassemia/E from 18 to 22 per cent, in hemoglobin H disease from 21 to 34 per cent and in beta-thalassemia major from 14-15 to 18-19 per cent. 2. The requirement of blood transfusions per year decreased significantly from 6 to 2 times in beta-thalassemia/E, 10 to 4-5 times in beta-thalassemia major and no transfusion was needed in hemoglobin H disease. 3. Postoperatively, there was a significant increase (p less than 0.05) in the liver size by the third year in beta-thalassemia/Hb E disease, and in the first year in beta-thalassemia major but the liver-size was decreased significantly by the fourth year in hemoglobin H disease. 4. Within 2 years postoperatively, the growth velocity in height kept up with their presplenectomy period in hemoglobin H and beta-thalassemia major (except two cases). The growth in weight kept up with their presplenectomy period in 40/49 cases (81.63%) in beta-thalassemia/Hb E but there was no change in the weight velocity in hemoglobin H and beta-thalassemia major. 5. There were 5 cases with immediate postoperative complications. Three cases had pneumonia, one case had septicaemia and one case had bleeding at the operative wound. Episodes of URI decreased in the post-operative period. Five cases of bacteremia developed within 6 years post-splenectomy.(ABSTRACT TRUNCATED AT 250 WORDS)