George D. Ludwig, M.D., F.A.C.P.; David Bilheimer, B.S.; Lynne Iverson, B.S.
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Erythropoietic protoporphyria (EPP), an inborn error of metabolism transmitted as a mendelian dominant trait, is characterized by increased erythrocyte protoporphyrin (EP) concentrations, solar sensitivity, and photocutaneous lesions. Increased photohemolysis of EPP red blood cells (RBC) by ultraviolet irradiation in vitro has been reported. We have found low plasma tocopherol concentrations in EPP, apparently due to its increased utilization as an antioxidant. The plasma tocopherol varied inversely with EP. Increased photohemolysis of vitamin E-deficient mouse RBCs by ultraviolet irradiation has been shown. These observations prompted the studies reported here.
Photohemolysis by ultraviolet, in vitro, was demonstrated in the propositi of five
Ludwig GD, Bilheimer D, Iverson L. Biochemical Mechanism of Photohemolysis of Erythrocytes in Erythropoietic Protoporphyria.. Ann Intern Med. ;66:1060. doi: 10.7326/0003-4819-66-5-1060_1
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Published: Ann Intern Med. 1967;66(5):1060.
Gastroenterology/Hepatology, Liver Disease.
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