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b5 - ebnalfady
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05-03-2008, 06:07 PM
To which of the following diseases is pyruvate kinase deficiency most similar clinically?
A. α-thalassemia B. β-thalassemia C. Glucose-6-phosphate dehydrogenase deficiency D. Hereditary spherocytosis E. Iron deficiency anemia
05-03-2008, 06:28 PM
Explanation:
The correct answer is C. Both pyruvate kinase deficiency and glucose-6-phosphate dehydrogenase deficiency are red cell enzyme deficiencies characterized clinically by long "normal" periods interspersed with episodes of hemolytic anemia triggered by infections and oxidant drug injury (antimalarial drugs, sulfonamides, nitrofurans). In both of these conditions, the cell morphology between hemolytic episodes is usually normal or close to normal. The α(choice A) and β(choice B) thalassemias, in their major forms, are characterized by persistent severe anemia. In the trait forms, they are charactertized by mild anemia. Hereditary spherocytosis (choice D) is characterized by intermittent hemolysis, but, unlike pyruvate kinase deficiency and glucose-6-phosphate dehydrogenase deficiency, oxidant drugs are not a specific trigger for hemolysis. Iron deficiency anemia (choice E) is characterized by chronic anemia with hypochromic, microcytic erythrocytes. |
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