The clinical history, laboratory, and necropsy findings in a patient with terminal jaundice and disordered porphyrin metabolism are presented. A retrospective diagnosis of erythropoietic protoporphyria is considered.
A 39-year-old spinster presented with a severe megaloblastic anemia after treatment of her epilepsy for eight years with the barbiturate, methophenobarbital, taken regularly and alone in therapeutic dose. This drug appears to have caused the anemia, which may have been conditioned by a folic-acid-poor diet. Hematologic remission followed the administration of folic acid, which she has since continued to take along with her previous dose of methophenobarbital. Disordered pigment metabolism, including methemalbuminemia, is reported and discussed.
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