Glucose phosphate spleen

Hemolytic anemia results from decreased RBC survival time due to destruction in the spleen or circulation. The most common etiologies are RBC membrane defects (e.g., hereditary spherocytosis), altered Hb solubility or stability (e.g., sickle cell anemia [see Chap. 34] and thalassemias), and changes in intracellular metabolism (e.g., glucose-6-phosphate dehydrogenase deficiency). Some drugs cause direct oxidative damage to RBCs (see Appendix 3). 

Oxidized, denatured hemoglobin forms aggregates, which can become attached to the inner surface of the red cell, known as Heinz bodies. This leads to damage to the red cell, which may result in direct destruction of the cell, which can be shown in vitro, or removal from the circulation by the spleen in vivo. When caused by Fava beans, the syndrome is known as Favism. As the deficient enzyme (glucose-6-phosphate dehydrogenase) is intrinsic to the red cell, exposure of such cells in vitro to suitable drugs will lead to cell damage and death. 

T14. Severity of Clinical Symptoms Due to Enzyme Deficiency The clinical symptoms of two forms of galactosemia—deficiency of galactokinase or of UDP-glucose galactose 1-phosphate uridylyltransferase— show radically different severity. Although both types produce gastric discomfort after milk ingestion, deficiency of the transferase also leads to liver, kidney, spleen, and brain dysfunction and eventual death. What products accumulate in the blood and tissues with each type of enzyme deficiency Estimate the relative toxicities of these products from the above information. 

Complete system contained (glucose-l- C) cerebroside (243,000 cpm 0,5 / mole), Cutscum, potassium phosphate buffer (pH 7.0) and supernatant liquid from either rat spleen or human spleen (70 mg protein). 

The main favism symptom is acute haemolytic anaemia, accompanied by high fever, jaundice and sweUing of the hver and spleen, as toxic pyrimidines oxidise the reduced form of glutathione in erythrocytes. Favism is manifested especially in individuals with low (usually hereditary) activity of the enzyme glucose 6-phosphate dehydrogenase in erythrocytes that reduces the oxidised form... 

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