title: The value of intravenous heme-albumin and plasmapheresis in reducing postoperative complications of orthotopic liver transplantation for erythropoietic protoporphyria.
abstract: Erythropoietic protoporphyria (EPP) is marked by a deficiency of ferrochelatase, which occurs in all cells and tissues, preventing effective conversion of proto porphyrin IX to heme and thereby blocking effective feedback inhibition of heme synthesis. The major source of the excess protoporphyrin is the bone marrow. Protoporphyrin IX may accumulate, with resultant toxicity chiefly of the marrow, skin, nervous system, and liver. Orthotopic liver transplantation (OLT) is, at present, the only adequate intervention for severe liver compromise secondary to protoporphyrin deposition, but it has been complicated by severe photosensitivity and polyneuropathy. Intravenous heme and plasmapheresis have been proposed but not previously reported as means to reduce the protoporphyrin burden before liver transplantation. We report a man with EPP who underwent preoperative heme-albumin administration and plasmaphereses that led to marked reductions in plasma and erythrocyte protoporphyrin levels. His OLT was uneventful, and he developed neither polyneuropathy nor exacerbation of photosensitivity.
authors: The Department of Medicine, The Liver, Biliary, Pancreatic Center, The University of Massachusetts/Memorial Health Care, Worcester 01655, USA.
authors: J H
authors: JH
authors: Reichheld
authors: E
authors: E
authors: Katz
authors: B F
authors: BF
authors: Banner
authors: I O
authors: IO
authors: Szymanski
authors: J R
authors: JR
authors: Saltzman
authors: H L
authors: HL
authors: Bonkovsky
mesh_terms: D000418:Albumins / Q000627:therapeutic use*; D006418:Heme / Q000627:therapeutic use*; D006801:Humans; D007275:Injections, Intravenous; D016031:Liver Transplantation / Q000009:adverse effects*; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D017121:Porphyria, Hepatoerythropoietic / Q000601:surgery*; D011183:Postoperative Complications / Q000517:prevention & control*
chemical_list: D000418:Albumins; D006418:Heme