Hereditary Spherocytosis: Indication of splenectomy and its long-term complications
Résumé
Hereditary spherocytosis (HS) is the most frequent cause of constitutional hemolytic anemia among Caucasians. It is due to a quantitative or qualitative defect of some of the proteins composing the skeleton of the red blood cell membrane. Destabilization of this membrane causes spherisation of the red blood cells and hemolysis, occurring mainly in the spleen. Splenectomy is consequently very efficient in this disease. Recent guidelines aimed at defining the place of splenectomy in HS: it is always indicated in severe cases, preferentially after the age of 6 years old; it should be discussed in mild presentations, and is never indicated in asymptomatic cases. Subtotal splenectomy may be an interesting option in children under 6 years old with severe presentation, but its place is not well defined yet. However, splenectomy is associated with long-term complications. Infectious complications are well known, and can be prevented with vaccination, education of the patient and his family, and with the systematic use of prophylactic antibiotics after splenectomy. It is noteworthy that the risk of overwhelming post-splenectomy infection persists all life long in splenectomized patients. There is also an increased thrombo embolic risk after splenectomy, including early splenic and portal thrombosis (often asymptomatic), and delayed arterial or venous events. A slightly increased risk of cancer has also been described in splenectomized patients, but this is still controversial, and has not been studied specifically in HS patients. All these complications should be taken into account when a splenectomy is discussed, but keeping in mind its clear benefits in symptomatic forms of HS.