Heme oxygenase deficiency, a rare condition disrupting heme metabolism, has only nine reported cases. We present a 3-year-old boy with dysmorphic facies, asplenia, and normal bilirubin levels despite ongoing hemolysis. Blood transfusions sustained hemoglobin while IV steroids managed inflammation.
Keywords: HMOX1 gene; asplenia; bilirubin synthesis; heme oxygenase deficiency.
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