Clinical and hematologic benefits of partial splenectomy for congenital hemolytic anemias in children.

OBJECTIVE

To assess the role of partial splenectomy for symptomatic children with various congenital hemolytic anemias.

SUMMARY BACKGROUND DATA: The use of total splenectomy for symptomatic children with congenital hemolytic anemias is restricted by concern of postsplenectomy sepsis. A partial splenectomy is an alternative procedure, although its utility remains incompletely defined.

METHODS

This longitudinal cohort study followed 25 symptomatic children with various congenital anemias who underwent partial splenectomy. Sixteen children had hereditary spherocytosis (HS), and nine children had other erythrocyte disorders. Outcome measures were clinical and laboratory hemolysis, splenic phagocytic and immune function, and splenic regrowth as measured by ultrasonography. Discrete parameters were compared using the Student test.

RESULTS

Partial splenectomy was successful in all 25 children, with minimal morbidity. Follow-up ranged from 7 months to 6 years (mean 2.3 +/- 1.5 years). Following surgery, children with HS had increased hemoglobin values, decreased reticulocyte and bilirubin levels, and preserved splenic function. Most children without HS had decreased symptoms of hypersplenism and splenic sequestration. Over time, variable rates of splenic regrowth were noted, although regrowth did not necessarily correlate with recurrent hemolysis.

CONCLUSIONS

In children with hereditary spherocytosis, a partial splenectomy appears to control hemolysis while retaining splenic function. In children with other congenital hemolytic anemias, a partial splenectomy appears to control symptoms of hypersplenism and splenic sequestration.