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Physiology · Blood and immunity: red cell oxidant defence
Sample itemA 25-year-old man develops dark urine and jaundice two days after starting primaquine. His smear shows bite cells, and supravital staining shows blue-purple inclusions attached to the membrane. Which defect makes his red cells vulnerable?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Too little NADPH to keep glutathione reduced, so haemoglobin is oxidised
Red cells depend on the pentose phosphate pathway for NADPH, which regenerates reduced glutathione to detoxify peroxides. When the first enzyme is deficient, oxidant drugs denature haemoglobin into Heinz bodies and macrophages remove the damaged cells, giving bite cells and haemolysis.
Option by option
A. Deficient spectrin, causing loss of the biconcave shape
Spectrin deficiency causes spherocytosis, with spherocytes rather than bite cells.
B. Inadequate ATP from glycolysis, causing failure of the cation pumps
ATP deficiency from pyruvate kinase loss gives chronic haemolysis, not drug-triggered episodes.
C. Unpaired globin chains precipitating within the red cell
Unpaired globin chains characterise thalassaemia and do not give an oxidant-triggered crisis.
D. Too little NADPH to keep glutathione reduced, so haemoglobin is oxidised (correct)
Correct. Without NADPH, glutathione cannot be regenerated and oxidant stress denatures haemoglobin.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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