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Red Cell Enzyme defects

Red Cell Enzyme defects. ATP required for energy (pumps / glucose) 23 Diphosphoglycerate (23 DPG) required to regulate Hb/O2 dissociation Reduction mechanisms for oxidized Globin -- G6PD dependant system Haem -- Methhb. reductase. Critical Enzymes/products.

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Red Cell Enzyme defects

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  1. Red Cell Enzyme defects • ATP required for energy (pumps / glucose) • 23 Diphosphoglycerate (23 DPG) required to regulate Hb/O2 dissociation • Reduction mechanisms for oxidized Globin -- G6PD dependant system Haem -- Methhb. reductase

  2. Critical Enzymes/products • G6PD • MethHb Reductase • Pyruvate Kinase • 23 DPG

  3. G6PD Deficiency • Globin spontaneously undergoes oxidation • Oxidized globin unstable ppts haemolysis • Oxidation stresses eg. Naphthalene Nitrofuradantin Antimalarials Analgesics Infections

  4. G6PD deficiency • Hexose monophosphate shunt products oxidation reduction • G6PD key enzyme • Deficiency leads to failure of reduction of oxydized globin • G6PD def. is relative and is cell age dependent • Older cells more sensitive to oxidant stress

  5. G6PD Deficiency • Frequency Multiple varients 11% of American Negros Mediterranean 1:1000 Jewish sect ---- 50%

  6. G6PD Deficiency • Inheritance X linked Lyon Hypothesis Random inactivation of X chromosome • Expression Normal with level >25% Cong nonspherocytic Haem Anaemia Drug induced Haem Anaemia Favism

  7. Other Anaemias • RBC Aplasia --- Acute/Chronic • Aplastic Anaemia --- All involved Idiopathic/2ary • Renal failure • Hypothyroidism • Chronic disease

  8. Anaemia of Renal Failure • Uremia --- Haemolysis Marrow supression • Haemorrhage • Reduced EPO • Phosphate retention --- O2 diss curve

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