tommyk
04-18-2004, 06:18 PM
Case: Pt with anemia from pyruvate kinase def. The anemia is primarily mild because ???
A) This AR disease develops an intrinsic hemolytic anemia with extravascular hemolysis. Pyruvate kinase catalyzes to form ATP. Without the ATPase dependent sodium potassium pump, RBCs lose their shape. BUT, substrates proximal to the point pyruvate kinase works is built up, and 2,3 bisphosphoglycerate concentration shifts the O2 dissociation curve to the right, making O2 delivery better.
A) This AR disease develops an intrinsic hemolytic anemia with extravascular hemolysis. Pyruvate kinase catalyzes to form ATP. Without the ATPase dependent sodium potassium pump, RBCs lose their shape. BUT, substrates proximal to the point pyruvate kinase works is built up, and 2,3 bisphosphoglycerate concentration shifts the O2 dissociation curve to the right, making O2 delivery better.