A Rare Hemoglobinopathy Presenting as Chronic Hyperbilirubinemia

HBsAg and anti HCV were negative. Since reticulocyte count was elevated and peripheral smear was suggestive of hemolytic anemia, she was worked up for the same and Hb electrophoresis was done which was suggestive of sickle beta thalassemia. Hydroxyurea, a myelosuppressive agent, is a prototype drug which increases fetal hemoglobin, reduces episodes of vaso-occlusive pain, acute chest syndrome and the need for blood transfusions and helps in prevention of chronic organ damage, leading to improved survival (5). King A, Shenoy S. Evidence-based focused review of the status of hematopoietic stem cell transplantation as treatment of sickle cell disease and thalassemia.