Liver transplantation for erythropoietic protoporphyria in Europe.

[erythropoietic protoporphyria]

Liver transplantation is an established lifesaving treatment for patients with severe protoporphyric liver disease , but disease recurrence in the graft occurs for the majority of recipients . Severe burn injuries may occur when protective light filters are not used with surgical luminaires . Motor neuropathy with an unclear pathogenesis is a frequent complication . We retrospectively studied 35 transplants performed for protoporphyric liver disease in 31 European patients between 1983 and 2008 . Most of the patients were male (61 .3 %), and the mean age at the time of primary transplantation was 39 years (range = 9 -60 years ). The overall patient survival rates were 77 % at 1 year and 66 % at 5 and 10 years . The overall rate of disease recurrence in the graft was 69 %. Forty -three percent of the patients experienced recurrence within a year , but this was often a transient finding that was associated with other graft complications . Phototoxic injuries due to surgical luminaires were seen in 25 .0 % of the patients who were not protected by filters , but these injuries were not seen in the 9 patients who were protected by filters . Significant motor neuropathies requiring prolonged ventilation complicated the postoperative course for 5 of the 31 patients (16 .1 %). Hematopoietic stem cell transplantation was performed for 3 patients to prevent graft loss due to disease recurrence . Prognostic markers are needed to identify patients prone to severe protoporphyric liver disease so that curative stem cell transplantation can be offered to select patients instead of liver transplantation .