HEMATOLOGIC STUDiES ON ERYTHROPOIETIC PORPHYRIA 763

With the technical assistance of Vicki Keefer E RYTHROPOIETIC porphyria is a rare inborn error of porphyrin metabolism; forty-two well documented cases have been described to date. Among the features common to this disorder is a hemolytic component of varying degree and severity. The precise nature of the hemolytic defect and its relationship to changes in other organs such as the liver and the spleen is not clear. The case reported here is that of an 8-year-old white male with a severe hemolytic anemia and thrombocytopenia who developed a folic acid deficiency during the course of investigation. Also emphasized in this report is the diffuse involvement in this disorder and the intracorpiiscular nature of the red cell defect.