A 32-year-old woman with known hereditary spherocytosis (HS) has Hb 6.8 g/dL, reticulocyte 12%, and jaundice. She has had three hemolytic crises in the past year requiring transfusion. Splenomegaly is confirmed on ultrasound. What is the definitive management?

• A Regular transfusion every 4 weeks
• B Splenectomy after appropriate vaccination ✓
• C Hydroxyurea therapy
• D Iron supplementation

Explanation

Splenectomy is the definitive treatment for symptomatic hereditary spherocytosis as the spleen is the primary site of spherocyte destruction. Patients should receive pneumococcal, meningococcal, and Haemophilus influenzae type b vaccinations at least 2 weeks before splenectomy. It does not correct the membrane defect but removes the site of extravascular hemolysis. Folic acid supplementation is adjunctive.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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