Severe liver failure in erythropoietic protoporphyria. Report of a case treated with liver transplantion

Severe liver failure is a rare complication of erythropoietic protoporphyri a (PEP), which is associated with a high rate mortality. Until now, 31 pati ents with this hepatic complication had underwent a liver transplantation, with a high rate of survival, but their long-term outcome is not well estab lished. We report the first case in Spain of PEP in 59 year-old, whose acute liver failure was treated with river transplantation, without postoperative compl ications. The patient is in good clinical condition 30 months later. Nevert heless during the first eleven months of follow-up the plasma levels of pro toporphyrin remained elevated, which was accompanied of biochemical and his tological evidence of relapse of the metabolic disease in the graft. Cases such as this stress the usefulness of liver transplantation, but also the n eed of more efficient measures to decrease the protoporphyrin levels before and after the transplant, in order to prevent hepatic and extrahepatic com plications in these patients.