Short- and long-term risks of splenectomy for benign haematological disorders: should we revisit the indications?

Splenectomy has represented a key treatment option in the treatment of many benign haematological diseases, including immune thrombocytopenia (ITP) and disorders associated with ongoing haemolysis (thalassaemia major and intermedia, sickle cell disease, and hereditary or acquired haemolytic anaemias). Improvements in surgical techniques have reduced perioperative complications and mortality. Preventive measures (new protein conjugate vaccines, antibiotic prophylaxis, and increased vigilance) are thought to greatly reduce the risk of overwhelming post-splenectomy infection (OPSI), although their implementation is inconsistent. Nevertheless, there is increasing documentation of the short- and long-term risks of splenectomy, which vary according to the underlying indication. Splenectomized patients are at increased risk of venous thromboembolism, particularly within the splenoportal system. The long-term thromboembolic risk is higher in haematological disorders associated with ongoing haemolysis, particularly in thalassaemia intermedia, which has led to a more conservative approach. In comparison, patients with ITP appear to be at lower risk of adverse effects of splenectomy, which maintains its place as the potentially most curative and safe second-line treatment. However, a splenectomy-sparing approach is also emerging for ITP, and recent guidelines recommend that this procedure is deferred until ≥ 12 months from ITP diagnosis, to allow sufficient time for possible remission.