PROPHYLACTIC SPLENECTOMY AND CHOLECYSTECTOMY IN MILD HEREDITARY SPHEROCYTOSIS - ANALYZING THE DECISION IN DIFFERENT CLINICAL SCENARIOS

Objectives. Patients with mild hereditary spherocytosis (HS), i.e. wit h haemolysis without anaemia, have an increased risk of gallstone form ation, erythroid aplasia and haemolytic crisis. Since the effect of pr ophylactic splenectomy on life expectancy has not been established, we conducted a decision analysis comparing prophylactic splenectomy and cholecystectomy with no surgery. Design. The available data on surgery and disease outcomes were modelled to estimate the effects of the dif ferent interventions on the quality-adjusted life expectancy. The earl y phase outcomes depicted surgery-related mortality and incorporated c ompliance and the adverse effects of prophylaxis against post splenect omy infections, The late phase outcomes were framed by a Markov cohort analysis. Results. For patients without gallstones, surgery was of no benefit. For those with gallstones the preferred strategies were foun d to be splenectomy and cholecystectomy before the age of 39 when asym ptomatic, and before 52 when accompanied by occasional biliary colic. Cholecystectomy alone proved to be the preferred strategy in older pat ients with occasional biliary colic. For patients of up to 52 years of age and candidates for cholecystectomy because of recurrent biliary c olic, the best strategy was to combine this procedure with splenectomy . Sensitivity analysis showed that the results were sensitive to the i ncidence of post cholecystectomy syndrome. Most remarkably an extreme sensitivity to compliance with post splenectomy infection prophylaxis was demonstrated. Conclusions. Our model suggested that combined proph ylactic splenectomy and cholecystectomy provide a substantial gain in quality-adjusted life expectancy for young patients and adults with mi ld HS and gallstones.