Pyridoxine-responsive anemia.

SI NCE THE ORIGINAL report by Harris et al.' in 1956, six additional I)ttients with anemia responsive to the administration of pyrinioxine have been described.27 With the exception of the case reported by Maier,2 tile features which have been common to this group of patients hiave I)een micro-cytic, hypochromic anemia, hyperferremia, hemosidlerosis and a partial or complete response of the anemia to the continued administration of relatively large amounts of pyridoxine. The condition has been termed " pyridoxine-responsive anemia " since in none of the subjects reported has there beell evidience suggestive of a dietary deficiency of pyridoxine. Furthermore, the anlounts of pyridoxine required to elicit a response have been in excess of the ordinary dietary requirement and, when the therapy has been discontinued!, relapses have been observed in spite of tile ingestion of a normal duet. Tile purpose of this paper is to report studies on two patients withi micro-cytic', hiypochromic anemia, hivperferremia, hemosiderosis, and a partii il hematologic response to pyridoxine. A preliminary report5 of one of these patleilts (M. Mc.) has been published. METHODS T11 e routine hematoh)gic methods used, including Heinz body stain, examination of urine and bone marrow for hemoside'rin, osmotic fragility, alkali resistant lmemnoglobimm, aiwr electrophoresis, serum bilirubin, urobilinogen estimation and urinary porpliobilinogemi have been described elsewhere.9 To identify reticulocytes in the presence of iron-containing Pappenheimner bodies, fresh smears were stained with brilliant cresyl blue followed l)y Prussian l)hme, amid were counter-stained with a mixture of basic fuchsin and neutral red. The details of this method are to he published.10 The method employed for the measurement of total serum copper was described 1)y Gubler et al.; 11 that for serum iron by Hamilton et al.; 12 for the total iron-binding capacity of the serum by Cartwright and \Vintrobe;'3 for xanthurenic acid l)y Glazer et al.;14 for starch block electrophoresis of hemoglobin by Kunkel et al.; 1 for free erythrocyte prOto-porphyrin and coproporphyrin by Schwartz and \Vikoff;16.1T or urinary coproporphyrin by Schwartz et al.'5 an adopted white i)oy of eight years of age was first seen in our clinic in March 1957. He lived in a state distant from Utah anti was seen and studied again in our clinic on two subsequent occasions, the summer of 1958 and the summer of 1960. Tlmi.s investzgati(nm. was .siipportt'd bb , a research grant (A-448.9) and a grathiate training grant (2A-5098) from the National Institute of Arthritis and …