Mitral valve replacement in Sickle C disease using intraoperative exchange transfusion.

Sickle C (SC) disease is a relatively uncommon hematologic disorder that poses special challenges when the patient requires a major surgical procedure. In particular, those who have a history of hemolytic crises require some type of intervention, usually homologous transfusion, to decrease the level of circulating hemoglobin S (HbS) and prevent intraoperative sickle crisis. We describe a 25-year-old man with SC disease and a history of multiple sickle cell crises who underwent mitral valve replacement using intraoperative exchange transfusion to decrease his HbS level from 53% to 7%.