The effect of phlebotomy as a treatment of Fabry disease.
Senescent erythrocytes are considered a major source of the ceramide trihexoside which accumulates in Fabry disease patients. We have evaluated weekly phlebotomy as a method to reduce the catabolic load imposed by the senescence of erythrocytes and thereby ceramide trihexoside in one Fabry patient; no change was observed in either plasma or urinary level of ceramide trihexoside. The implications of this observation are discussed with regard to glycolipid metabolism in man.
D001815:Bloodletting; D004905:Erythrocyte Aging; D004912:Erythrocytes / Q000378:metabolism; D000795:Fabry Disease / Q000097:blood / Q000628:therapy*; D006801:Humans; D008297:Male; D014281:Trihexosylceramides / Q000097:blood
D014281:Trihexosylceramides