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.
Eefsen
Martin
M
Department of Hepatology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. Martin@Eefsen.dk
Rasmussen
Allan
A
Wulf
Hans Christian
HC
Brock
Axel
A
Hansen
Bent Adel
BA
D000328:Adult; D004912:Erythrocytes / Q000378:metabolism; D006801:Humans; D017093:Liver Failure / Q000209:etiology; D016031:Liver Transplantation; D008297:Male; D009994:Osmolar Concentration; D010787:Photosensitivity Disorders / Q000209:etiology; D010956:Plasmapheresis; D011300:Preoperative Care; D046351:Protoporphyria, Erythropoietic / Q000097:blood / Q000150:complications / Q000628:therapy*; D011524:Protoporphyrins / Q000097:blood; D006435:Renal Dialysis; D012709:Serum Albumin; D016896:Treatment Outcome
D011524:Protoporphyrins; D012709:Serum Albumin; C028025:protoporphyrin IX