What is the treatment of choice for severe hereditary spherocytosis?

What is the treatment of choice for severe hereditary spherocytosis?

• A. Iron chelation
• B. Folic acid supplementation
• C. Splenectomy
• D. RBC transfusion therapy

Answer: (D)

Hereditary spherocytosis causes ongoing hemolysis mainly in the spleen because the defective red cells are preferentially trapped and destroyed there. Removing the spleen—the site of most destruction—greatly reduces hemolysis and increases the lifespan of red cells, making splenectomy the best long-term treatment for severe cases. In children this is typically done after about five years of age to lower the risk of overwhelming post-splenectomy infections; vaccines against encapsulated organisms and appropriate antibiotics are part of the preventive plan. Supportive measures like folic acid help with increased red cell production but do not stop the underlying destruction. Red blood cell transfusions may be necessary for acute severe anemia or crises, but they don’t address the root problem and are not a curative long-term strategy. Splenectomy provides a durable improvement in anemia and hemolysis, which is why it’s the treatment of choice for severe hereditary spherocytosis.

Hereditary spherocytosis causes ongoing hemolysis mainly in the spleen because the defective red cells are preferentially trapped and destroyed there. Removing the spleen—the site of most destruction—greatly reduces hemolysis and increases the lifespan of red cells, making splenectomy the best long-term treatment for severe cases. In children this is typically done after about five years of age to lower the risk of overwhelming post-splenectomy infections; vaccines against encapsulated organisms and appropriate antibiotics are part of the preventive plan.

Supportive measures like folic acid help with increased red cell production but do not stop the underlying destruction. Red blood cell transfusions may be necessary for acute severe anemia or crises, but they don’t address the root problem and are not a curative long-term strategy. Splenectomy provides a durable improvement in anemia and hemolysis, which is why it’s the treatment of choice for severe hereditary spherocytosis.