Successful Management of Transfusion-Dependent Unstable Hemoglobin Perth with Splenectomy: First Report from Iran and Literature Review.

Unstable hemoglobin (Hb) variants are a rare cause of congenital non-spherocytic hemolytic anemia, characterized by hemoglobin instability and Heinz body formation. Diagnosis is often challenging and requires a high index of suspicion, supported by targeted laboratory investigations. We report the first documented case of an unstable hemoglobin variant identified as hemoglobin Perth (also known as Hb Abraham Lincoln) from Iran in a patient who was transfusion-dependent since early childhood. At 10 years of age, he presented with acute cholecystitis and cholelithiasis, accompanied by profound direct hyperbilirubinemia. He underwent cholecystectomy and splenectomy, which resulted in significant clinical improvement, including transfusion independence and stabilization of hemoglobin levels. This case is particularly noteworthy for the exceptionally high levels of direct bilirubin unreported in previous cases of Hb Perth, as well as the successful therapeutic outcome achieved following splenectomy.