title: Hemolytic anemia in protoporphyria: possible precipitating role of liver failure and photic stress.
abstract: Hemolytic anemia is not a common clinical feature in protoporphyria. In this report, we describe two patients in whom we have encountered severe hemolytic anemia. Both individuals had advanced hepatic disease as a complication of their porphyria and were undergoing orthoptic liver transplantation. The onset of hemolysis appeared to be related to the development of liver disease, and in both cases, the operative procedure acutely exacerbated the red cell destructive process. We suggest that liver involvement in protoporphyria may unmask hemolytic anemia, and that red cells in these individuals are hypersensitive to photooxidative stress, such as might occur during a prolonged operative procedure.
authors.affiliation: Department of Medicine, University of Minnesota Medical School, Minneapolis.
authors.forename: N S
authors.initials: NS
authors.lastname: Key
authors.forename: J M
authors.initials: JM
authors.lastname: Rank
authors.forename: D
authors.initials: D
authors.lastname: Freese
authors.forename: J R
authors.initials: JR
authors.lastname: Bloomer
authors.forename: D E
authors.initials: DE
authors.lastname: Hammerschmidt
mesh_terms: D000293:Adolescent; D000743:Anemia, Hemolytic / Q000209:etiology*; D004910:Erythrocyte Membrane / Q000378:metabolism; D004912:Erythrocytes / Q000528:radiation effects; D006801:Humans; D007432:Intraoperative Period; D008027:Light / Q000009:adverse effects*; D008107:Liver Diseases / Q000150:complications*; D016031:Liver Transplantation; D008297:Male; D008315:Malondialdehyde / Q000097:blood; D008875:Middle Aged; D011164:Porphyrias / Q000150:complications*
chemical_list: D008315:Malondialdehyde