2 CASES OF OXALOSIS AS A CAUSE OF RESISTANCE TO RECOMBINANT-HUMAN-ERYTHROPOIETIN

Type I primary hyperoxaluria is a rare autosomal recessive metabolic d isease caused by a deficiency of hepatic peroxisomal alanine:glyoxylat e aminotransferase. It is characterized by the accumulation of calcium oxalate within numerous tissues, especially in the kidneys and bone m arrow. We report on 2 such cases discovered in patients with end-stage renal failure. Uremic patients with primary oxalosis often present wi th severe anemia. Severe anemia was, in fact, noted-which appeared unr esponsive to high doses of rHuEPO-and red cell transfusions were neede d frequently. Other causes of resistance to rHuEPO were excluded: iron depletion, vitamin B12 or folate deficiency, aluminum overload, infla mmatory process, malignancy, and infection. Bone marrow infiltration b y oxalate crystals represents a major limiting factor of this disease in its response to rHuEPO.