Acquired Porphyria Cutanea Tarda, Primary Refractory Anemia, and Hepatic Siderosis: Report of a Case

ACQUIRED porphyria cutanea tarda (PCT) is a disorder of hepatic porphyrin metabolism occurring usually in middle-aged alcoholics, manifested clinically by cutaneous photosensitivity and characterized biochemically by the excessive excretion of uroporphyrin in the urine and stool and the presence of uroporphyrin in the plasma. Hypersideremia and hemosiderosis are frequent findings in PCT and may be related to excessive dietary iron and augmented intestinal iron absorption associated with chronic alcoholism. 1-6 The observation that repeated phlebotomies are followed by a reduction in uroporphyrin excretion and marked clinical improvement in patients with PCT suggests that iron may play an important role in uroporphyrin synthesis. 4,7 In 1963, two cases of hemolytic anemia associated with PCT were reported from France. 8,9 Shortly afterward, the first known case of PCT associated with a refractory anemia was reported by Kyle et al. 10 The combination of PCT and primary refractory anemia is not only a rare occurrence but one