BENIGN EXTREME HYPERBILIRUBINEMIA IN A 9 YEAR-OLD GIRL WITH SICKLE-THALASSEMIA AND THE PROBABLE ROLE OF HBF IN PREDICTING THE OUTCOME

Hepatic dysfunction is a frequent manifestation in patients with sickle cell anemia. It is usually a multifactorial process. A rare benign form of extreme hyperbilirubinemia, presumably due to intrahepatic sickling, may be the cause. We report a 9 year old girl with sickle-thalassemia hemoglo binopathy, presenting with profound jaundice. Sickle cell disease is often mild in the Iranian population due to relatively higher levels of HbF, suggesting that the βs gene is associated with a gene capable of producing high levels of HbF. Moreover, sickle thalassemia disease is generally milder than sickle cell disease. In this patient, the previous electrophoresis had shown a relatively high HbF level (34.3%). This may account for the benign course of hyperbilirubinemia and no need for blood transfusion in this case, despite the majority of previous reports