A 24-year-old man of Mediterranean descent develops acute hemolysis 48 hours after starting primaquine for malaria prophylaxis. Peripheral smear shows blister cells and Heinz bodies after supravital staining. Which red cell enzyme deficiency is responsible?
- A Glucose-6-phosphate dehydrogenase deficiency ✓
- B Pyruvate kinase deficiency
- C Hexokinase deficiency
- D Glucuronyl transferase deficiency
Correct answer: A. Glucose-6-phosphate dehydrogenase deficiency
Explanation
G6PD deficiency causes oxidative hemolysis triggered by primaquine, fava beans, or infection. Peripheral smear shows bite (blister) cells and Heinz bodies. Pyruvate kinase deficiency causes chronic hemolysis without oxidative triggers. Hexokinase deficiency is extremely rare. Glucuronyl transferase deficiency causes unconjugated hyperbilirubinemia (Gilbert or Crigler-Najjar), not hemolytic anemia.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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