BENEFITS OF CHRONIC PLASMAPHERESIS AND INTRAVENOUS HEME-ALBUMIN IN ERYTHROPOIETIC PROTOPORPHYRIA AFTER ORTHOTOPIC LIVER TRANSPLANTATION12

Abstract

Erythropoietic protoporphyria (EPP) is characterized by a deficiency of ferrochelatase the final enzyme of the heme biosynthetic pathway. Patients with EPP may overproduce protoporphyrin IX, chiefly in developing erythrocytes. In some, protoporphyrin accumulates and causes toxicity, particularly to the skin and liver. Orthotopic liver transplantation (OLT) treats the severe liver disease that sometimes occurs in EPP; however, it does not correct the underlying metabolic disorder. We recently reported a patient with EPP who was improved with plasmapheresis and i.v. heme-albumin before OLT. Subsequently he developed histological and biochemical evidence of recurrent hepatotoxicity from protoporphyrin in the graft liver. We now report successful treatment of the patient with additional plasmapheresis and heme-albumin with improvement of hepatic histological and biochemical abnormalities. We conclude that plasmapheresis and heme-albumin are of benefit in EPP complicated by hepatotoxicity before and after liver transplantation.