Clarification needed for PK deficiency

[DarlynVMD]

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So now I'm totally confused, unless I'm missing a major point... hopefully someone can clarify this confusion. In RR Biochem, Goljan & Pelley state that in PK deficiency, there is inadequate ATP to maintain the ion pumps in the RBC membrane, and this results in loss of H20. In Kaplan Biochemistry, they state that the erythrocytes lose their characteristic biconcave shape, a direct result of the decrease in ATP. In RR Path, Goljan states the the chronic lack of ATP damages the RBC membrane, resulting in the dehydration of the RBC (echinocytes). So far so good, they all agree. But then... Kaplan Biochemistry, goes on to say that the cells swell and lyse as a direct result of the decreased pumping activity by the Na/K pump..... This too make sense, hence the chronic hemolysis associated with the dz. So my q is, and I might have the answer right in front of me, but it's either I'm too tired, or I just can't see it.... do you have dehydration of the RBC b/c of the loss of H20 and, with subsequent decrease in ion pump activity, swelling and lysis of the cells? It's obvious that the most important feature here is the hemolytic anemia, but what is, therefore, the significance of the dehydrated RBCs, which ultimately produce echinocytes?

Sorry.. I just think I'm tired now.

 

[LUBDUBB]

So now I'm totally confused, unless I'm missing a major point... hopefully someone can clarify this confusion. In RR Biochem, Goljan & Pelley state that in PK deficiency, there is inadequate ATP to maintain the ion pumps in the RBC membrane, and this results in loss of H20. In Kaplan Biochemistry, they state that the erythrocytes lose their characteristic biconcave shape, a direct result of the decrease in ATP. In RR Path, Goljan states the the chronic lack of ATP damages the RBC membrane, resulting in the dehydration of the RBC (echinocytes). So far so good, they all agree. But then... Kaplan Biochemistry, goes on to say that the cells swell and lyse as a direct result of the decreased pumping activity by the Na/K pump..... This too make sense, hence the chronic hemolysis associated with the dz. So my q is, and I might have the answer right in front of me, but it's either I'm too tired, or I just can't see it.... do you have dehydration of the RBC b/c of the loss of H20 and, with subsequent decrease in ion pump activity, swelling and lysis of the cells? It's obvious that the most important feature here is the hemolytic anemia, but what is, therefore, the significance of the dehydrated RBCs, which ultimately produce echinocytes?

Sorry.. I just think I'm tired now.

cells that have trouble maintaining the cell membrane integrity can lyse -eg.G6PDH or become "Thorny" RBC's eg. abetalipoproteinemia (marked dec in Vit E) and PK deficiency.