The value of intravenous heme-albumin and plasmapheresis in reducing postoperative complications of orthotopic liver transplantation for erythropoietic protoporphyria

Erythropoietic protoporphyria (EPP) is marked by a deficiency of ferrochela tase, which occurs in all cells and tissues, preventing effective conversio n of proto porphyrin IX to heme and thereby blocking effective feedback inh ibition 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 t ransplantation (OLT) is, at present, the only adequate intervention for sev ere liver compromise secondary to protoporphyrin deposition, but it has bee n complicated by severe photosensitivity and polyneuropathy, Intravenous he me and plasmapheresis have been proposed but not previously reported as mea ns to reduce the protoporphyrin burden before liver transplantation. We rep ort a man with EPP who underwent preoperative heme-albumin administration a nd plasmaphereses that led to marked reductions in plasma and erythrocyte p rotoporphyrin levels. His OLT was uneventful, and he developed neither poly neuropathy nor exacerbation of photosensitivity.