Aceruloplasminemia is a rare hereditary disorder characterized by severe iron overload in all organs, but with reduced serum iron. Ceruloplasmin is a ferroxidase necessary for tlie release of intracellular iron, intracellular ferrous iron being oxidized for subsequent transfer to plasma apotransferrin. Ceruloplasmin deficiency thus leads to intracellular iron overload which is not accessible to the plasma, and hence to erythropoiesis and to phlebotomy. A forty-two year old female Caucasian presented with dizziness, fatigue, elevated blood sugar and a ferritin level of 989 ug/L (normal 25-235). Hb was 12.9 G/L, MCV 82.7, serum iron 3 umol/L (normal 12-23umol/L ), iron binding capacity 57umol/L (normal 45-80umol/L ). Liver and bone marrow biopsies reveal 4+ iron overload. Thirty-five phlebotomies (500ml), once every 14 days, reduced ferritin to between 400-600 ug/L, but the patient became anemic with hemoglobin of 8.8 and MCV of 74.2. Repeat marrow and liver biopsies showed no change in the iron overload. Ceruloplasmin was not detectable in the serum, serum copper was 1.1 umol/L (normal 11.0-22.0 umol/L). DNA studies demonstrated homozygous change in the Ceruloplasmin gene, exon 4, 215 Arg (CGA) to Stop (TGA). MRI showed marked cerebral iron overload. Canadian Blood Services supplies plasma from donors with the best levels of Ceruloplasmin, and the patient is given one unit of plasma weekly with phlebotomies, done two out of every three weeks. Ceruloplasmin is said to have a half-life of about 5 days. Although it is detectable in the patient's serum after plasma transfusion, we have not been able to estimate the half-life. She has presently had sixty-four phlebotomies and her Ferritin is 322, MCV 84.8 and Hemoglobin 11.5. Updates on clinical progress, MR], liver, marrow, and genetic studies will be presented. This would be the first report of an adult North American patient with this disorder, and the first patient treated.