Thalassemia major is a rare inherited anemia, and affected children require blood transfusions every 2-4 weeks to survive. Repeated blood transfusions lead to a build-up of toxic levels of iron in the body, causing organ damage and premature death, primarily due to iron-induced heart disease. Deferiprone is one of a few drugs that are routinely used in medicine for the treatment of iron overload in thalassemic patients. This drug is usually administered daily at high doses (50-100 mg/kg) with a very low toxicity. Agranulocytosis is the most serious side effect of deferiprone, with a reported incidence of 0.6 per 100 patient-years. We document an illustrated case report of necrotizing gingivostomatitis, an oral manifestation of agranulocytosis secondary to deferiprone use involving the gingiva and palatal mucosa of a thalassemia major patient. Various causes of precipitation of agranulocytosis in these patients and a possible relationship of necrotizing gingivostomatitis with deferiprone are highlighted in this case report.
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