Term
| Identify the two main pathways by which erythrocytes catabolize glucose |
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Definition
(1)Glycolysis
(2)Hexose monophosphate shunt pathway |
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Term
| Explain the role of erythrocyte enzymes in maintaining the cell’s integrity |
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Definition
| They protect the cell from oxidant damage and provide the cell with energy. GSH maintains Hb in reduced functional state and G6PD maintains glycolysis to provide energy to the cell |
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Term
| Describe how deficiencies in these enzymes lead to anemia. |
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Definition
| Deficiencies in these enzymes compromise the integrity of the cells membrane. GSH deficiencies cause oxidation of hemoglobin, formation of Heinz bodies, which the spleen removes and leads to extravascular hemolysis. G6PD deficiencies cause decreased ATP and impaired cation pumping which increases osmotic fragility and causes hemolysis |
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Term
| Identify the most common erythrocyte enzyme deficiency |
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Definition
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Term
| Describe the inheritance pattern for glucose-6-phosphate dehydrogenase (G6PD). |
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Definition
| X-linked genetic disorder, more likely to affect males, will only manifest in females is she is homozygous. |
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Term
| Explain how the diagnosis of G6PD deficiency is made |
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Definition
| Demonstration of an increased G6PD activity by performing esoteric assays 2-3 months after hemolytic episode |
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Term
| Recognize the erythrocyte morphology in a Romanowsky-stained blood smear associated with G6PD deficiency |
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Definition
| Polychromasia, sphereocytes, small hypochromic cells, schistocytes, bite cells, blister cells, retics, and increased leukocytes, Heinz bodies |
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Term
| Identify common compounds that induce anemia in G6PD deficiency |
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Definition
| Antimalarial drugs, primaquine |
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Term
| Explain the function of glutathione in maintaining cellular integrity. |
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Definition
| GSH protects RBC from oxidant damage, maintains Hb in the reduced functional state, and preserves vital cellular enzymes |
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Term
| Defects in glycolytic pathways |
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Definition
| show extravascular hemolysis because the Heinz bodies precipitate in the cell because they are being oxidized, so the spleen removes them and eventually there is no more membrane and the cell hemolysis in the spleen |
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Term
| Defects in hexose-monophosphate shunt pathways |
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Definition
| show extravascular hemolysis because of the precipitation of Heinz bodies which is removed by the spleen |
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Term
| Acute, acquired HA- clinical findings |
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Definition
| hemolytic attacks after infections or expose to drugs, variable hemolysis due to degree of oxidant stress and sex and acute intravascular hemolysis due to drug-induced oxidation |
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Term
| Favism- clinical findings |
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Definition
| sudden, severe hemolytic episode, usually affect kids, malaise, lethargy, nausea, vomiting, abdominal pain, chills, tremors, fever, hemoglobinuria, jaundice |
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Term
| Congenital nonspherocytic HA-clinical findings |
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Definition
| clinical findings: class I G6PD variants: chronic hemolysis, compensation, mild anemia, reticulcytosis, |
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Term
| Congenital nonspherocytic HA-lab findings |
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Definition
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Term
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Definition
| severe hyperbilirubinemia, potential for kerniceterus |
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Term
| variants of G6PD lab findings before attack |
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Definition
| no anemia, normal blood until hemolytic attack |
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Term
| Lab findings after attack |
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Definition
| polychrome, sphereocytes, small hypochromic cells, schistocytes, bite cells, blister cells, retics, and leukocytes |
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Term
| Diagram the reaction catalyzed by pyruvate kinase |
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Definition
| Ending step in glycolysis: 2 phosphoenolpyruvate -> 2 pyruvate (ADP --> ATP) |
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Term
| explain how a defect of PK can cause hemolysis. |
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Definition
| If PK is deficient then intermediate of glycolysis will build up, the rappaport leubering shunt will produce more 2, 3-BPG |
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Term
| Recognize erythrocyte morphology associated with pyruvate kinase deficiency |
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Definition
| Normochromic/normocytic, retics, echinocytes, nRBCs |
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