Pediatrics · Pediatric Hematology and Oncology

A 4-year-old boy with hereditary spherocytosis has compensated hemolysis with hemoglobin 9.5 g/dL and symptomatic pigment gallstones. His parents ask why splenectomy is being deferred. The main reason is prevention of which complication?

  • A Recurrence of cholelithiasis
  • B Postoperative portal vein thrombosis
  • C Overwhelming post-splenectomy infection
  • D Transfusion-related iron overload
Correct answer: C. Overwhelming post-splenectomy infection

Explanation

Splenectomy cures the anemia of hereditary spherocytosis but removes the main defense against encapsulated organisms. Children under 5 to 6 years carry a much higher lifetime risk of overwhelming post-splenectomy infection from Streptococcus pneumoniae, so surgery is deferred until at least 5 to 6 years of age, with vaccination beforehand. Portal vein thrombosis and recurrence of stones are real considerations but do not dictate this specific age threshold.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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