Erythropoietic protoporphyria and pretransplantation treatment with nonbiological liver assist devices

Erythropoietic protoporphyria (EPP) is a disease of the heme metabolism due to a deficiency of ferrochelatase, leading to accumulation of protoporphyrin (PPIX) in the erythrocyte (red blood cell [RBC]). The major clinical manifestation in EPP is photosensitivity; however, in a small number of patients liver failure is a significant complication and liver transplantation is the only treatment option. Damage to both abdominal skin and organs occurs when exposed to operating light; however, this problem can be ameliorated by the use of filters that block the transmission of light with wavelength below 470 nm. A more unusual but very serious complication postoperatively is severe motor neuropathy, with few or no known acute available precautions. An effective treatment option is needed to manage EPP crises and to prevent complications after liver transplantation. We successfully treated a patient with EPP-induced liver failure with the molecular adsorbents recirculating system (MARS®) and Prometheus® in independent sessions. Following treatment with MARS® we found a 9.1% reduction of the RBC-PPIX concentration and a 5.9% reduction after treatment with the Prometheus® system. Plasmapheresis made a reduction in RBC-PPIX concentration of 0.8%. Following treatment sessions with MARS® and Prometheus®, the clinical condition was markedly improved and orthotopic liver transplantation was performed without further complications. In conclusion, extracorporeal therapy with MARS® or Prometheus® seems to be efficient in reducing RBC-PPIX concentration in comparison to plasma exchange. Liver Transpl, 2007. © 2007 AASLD.