What are indications for splenectomy in a patient with hereditary spherocytosis (HS)?

By William Aird

  • Symptomatic hemolytic anemia
  • Presence of gallstones
  • Large reduction in exercise tolerance
  • Growth retardation
  • Skeletal changes or leg ulcers due to HS
  • Extramedullary hematopoietic tumors
  • Vascular compromise of vital organs in older patients
  • Splenic infarct with pain or early satiety from splenomegaly

Consider delaying splenectomy until > 6 years old if possible.

Guideline recommendations:

Recommendations regarding splenectomy in hereditary hemolytic anemias by Splenectomy in Rare Anemias Study Group (2017):

2012 British Committee Standards in Haematology (BCSH) expert guideline on diagnosis of hereditary spherocytosis:

Patients should be selected for splenectomy on the basis of their clinical symptoms and presence of complications such as gallstones, not simply on the basis of the diagnosis alone (grade 2 recommendation, grade C evidence). Splenectomy should be performed in children with severe HS, considered in those who have moderate disease, and should probably not be performed in those with mild disease. A careful history is important in those with moderate to mild disease to establish if there is evidence suggesting reduced exercise tolerance; the metabolic burden of increased marrow turnover may be considerable. Where there is a family history, the benefit of splenectomy in other individuals may help determine whether to proceed.