Risks and benefits of splenectomy versus no splenectomy for hereditary spherocytosis--a personal view.
Splenectomy is indicated in hereditary spherocytosis to relieve symptoms due to anaemia or splenomegaly, reverse growth failure or skeletal changes due to over-robust erythropoiesis, and prevent recurrent gallstones. A life-long risk of bacterial infection has been recognised for many years as a concomitant cost of splenectomy. The scope of this risk has expanded to include a number of organisms beyond the triad of pneumococcus, meningococcus, and haemophilus influenzae. Recently, it has been demonstrated that splenectomy also confers a significant risk of delayed adverse vascular events in patients with hereditary spherocytosis, just as it does in patients undergoing splenectomy for other indications. Further, these same studies demonstrated a benefit of avoiding splenectomy: hereditary spherocytosis patients with a spleen have significantly fewer adverse vascular events than unaffected family members, probably because of the protective effect of chronic, mild anaemia. Accordingly, this review marshals the evidence favouring a conservative approach to splenectomy in spherocytosis.
Schilling
Robert F
RF
School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI 53706, USA. rfsmd@medicine.wisc.edu
D000328:Adult; D001424:Bacterial Infections / Q000150:complications; D002648:Child; D006801:Humans; D009203:Myocardial Infarction / Q000150:complications; D018579:Patient Selection; D012306:Risk; D013103:Spherocytosis, Hereditary / Q000150:complications / Q000601:surgery*; D013156:Splenectomy; D016896:Treatment Outcome