Risks and benefits of splenectomy versus no splenectomy for hereditary spherocytosis--a personal view

Br J Haematol. 2009 Jun;145(6):728-32. doi: 10.1111/j.1365-2141.2009.07694.x. Epub 2009 Apr 15.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Bacterial Infections / complications
  • Child
  • Humans
  • Myocardial Infarction / complications
  • Patient Selection
  • Risk
  • Spherocytosis, Hereditary / complications
  • Spherocytosis, Hereditary / surgery*
  • Splenectomy*
  • Treatment Outcome