Long-Term Follow Up Of The Beneficial Effects And Of Issues In Subtotal Splenectomy For Hereditary Spherocytosis

Total splenectomy is recommended in symptomatic cases of hereditary spherocytosis (HS) to reduce the severity of anemia but exposes patients to long-term infectious or thrombotic complications. Alternative strategies such as subtotal splenectomy (STS) have been developed, principally for children under the age of 6 with severe HS, who are not eligible to total splenectomy because of the high infectious risk and for older patients with mild HS complaining of chronic discomfort. Since our original report, several groups have shown that STS reduced the hemolytic rate, increased the red cell lifespan while maintaining an efficient splenic phagocytic function but the extent of follow-up has been limited. In order to define the long-term benefits of STS, we report here an update of our series that includes 90 patients who underwent STS at the Bicetre hospital between 1985 and 2013, with a median-follow-up of 9.3 years (range 1-23 years). Two groups were defined on the basis of the disease phenotype: Group A included 42 patients for whom STS was performed because of severe/intermediate HS (transfusion requirement or hemoglobin (Hb) level In summary, our results show that in the long-term, STS resulted in a decrease in the hemolytic rate in HS. In children under the age of six years with severe HS, STS decreased the transfusion rate and increased the hemoglobin to a level compatible with normal growth while retaining splenic function. However, half of these patients will require total splenectomy, but at an age where it will be much safer. In milder HS, STS removed discomfort related to the high hemolytic rate whithout exposing the patients to the risks of a total splenectomy. Disclosures No relevant conflicts of interest to declare.