Sideroblastic Anemia with Dermal Photosensitivity and Greatly Increased Erythrocyte Protoporphyrin

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Abstract
Sideroblastic anemia was found in an elderly man in association with dermal photosensitivity, a greatly increased erythrocyte free protoporphyrin content and reduced erythrocyte catalase activity. Markedly reduced heme synthetase activity was found in the patient's reticulocytes. Since the activity of mixtures of the patient's cells with cells from a patient with hemolytic anemia was greater than the sum of the components of the mixture, the patient may have lacked an essential cofactor. The clinical, hematologic and biochemical manifestations of disease in this case appear to be consequences of this defect.

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