Epoetin for Severe Anemia in Hepatoerythropoietic Porphyria

To the Editor: Hepatoerythropoietic porphyria is a rare autosomal recessive disorder of heme biosynthesis caused by a deficiency of uroporphyrinogen decarboxylase. Excessive accumulation of photoactive porphyrins in the skin, bone marrow, and peripheral blood results in photodermatitis, secondary skin infections, and “intractable” anemia.1 We report the successful treatment of life-threatening anemia with recombinant human erythropoietin (epoetin) in a patient with late-onset hepatoerythropoietic porphyria. A 68-year-old white man (Karnofsky score, 20)2 with a 36-year history of porphyria and anemia was admitted for severe heart failure (left ventricular ejection fraction, 30 percent). Disfiguring scarring with inflamed erosions was present on sites exposed . . .