Liver transplantation for erythropoietic protoporphyria: report of a case with medium-term follow-up

• Published in: Digestive and liver disease Vol. 32; no. 9; pp. 799 - 802
• Main Authors: Leone, N., Marzano, A., Cerutti, E., Actis, G.C., Marchesa, P.E., David, E., Salizzoni, M., Rizzetto, M.
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ltd 01-12-2000
• Subjects: Adult; Biopsy, Needle; cirrhosis; erythropoietic protoporphyria; Follow-Up Studies; Graft Survival; Humans; Liver Cirrhosis - diagnosis; Liver Cirrhosis - etiology; Liver Cirrhosis - surgery; Liver Transplantation; Male; Porphyria, Hepatoerythropoietic - complications; Porphyria, Hepatoerythropoietic - diagnosis; Risk Assessment; Severity of Illness Index; syncytial acute hepatitis; Time Factors; erythropoietic protoporphyria; syncytial acute hepatitis; cirrhosis; liver transplantation
• ISSN: 1590-8658; 1878-3562
• DOI: 10.1016/S1590-8658(00)80359-X

A case of liver transplantation is described in a 35-year-old male with hepatic failure due to erythropoietic protoporphyria. Data regarding protoporphyrin levels in erythrocytes and faeces, before and after transplantation, seem to indicate that, in this case, protoporphyrin overproduction was, in part, due to liver synthesis. Four years after surgery, the patient is completely free of skin photosensitivity. Liver function tests are normal and there are no significant protoporphyrin deposits in the new liver. However, recurrence of the disease in the long-term cannot be excluded, since erythrocyte protoporphyrin levels have remained elevated after liver transplantation.