Estimating the Risk for Sepsis after Splenectomy in Hereditary Spherocytosis

Since the alarming report by King and Schumacher [1] in 1952, it has been evident that some asplenic persons are at increased risk for life-threatening and fatal bacterial infections. Because all of the patients described in the 1952 report were children, it was briefly thought that this risk might be higher in children only. However, subsequent reports [2-6] have provided convincing evidence that splenectomy carries a substantial risk for sepsis in adults as well; adults should be instructed in prevention. I have been unable to find a quantitative estimate of the risk for fatal fulminant sepsis in persons who have had a splenectomy for hereditary spherocytosis. Although an extensive review by Singer [4] found a 2.2% mortality from sepsis after splenectomy for hereditary spherocytosis, such a review cannot estimate fatalities per year at risk. As a result of my long-term interest in this disorder [7, 8], I have access to numerous persons who have had splenectomies for hereditary spherocytosis, and I surveyed them to estimate this risk. Two hundred twenty-six persons lived a total of 5461 years after splenectomy. Four of them died; the estimated mortality rate is thus 0.73 per 1000 years.