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Multiple Choice

A CBC shows echinocytes (burr-like cells) and supravital staining reveals multiple dark inclusions attached to RBC membranes. LDH is elevated and DAT is negative. Which enzyme deficiency is most likely?

This picture fits nonimmune hemolytic anemia from a red cell enzyme defect that destabilizes the membrane. Pyruvate kinase deficiency reduces ATP production in red cells, which weakens the membrane–cytoskeleton interactions and causes the cell to become spiculated, producing echinocytes (burr cells) on the smear. The elevated LDH confirms ongoing hemolysis, and the negative direct antiglobulin test argues against autoimmune causes. The supravital stain showing dark inclusions attached to the membrane reflects membrane-bound hemoglobin aggregates that can occur in red cells with membrane instability and nonimmune hemolysis. The other options describe conditions with different smear features or immune-mediated destruction (positive DAT), making them less consistent with this pattern.

This picture fits nonimmune hemolytic anemia from a red cell enzyme defect that destabilizes the membrane. Pyruvate kinase deficiency reduces ATP production in red cells, which weakens the membrane–cytoskeleton interactions and causes the cell to become spiculated, producing echinocytes (burr cells) on the smear. The elevated LDH confirms ongoing hemolysis, and the negative direct antiglobulin test argues against autoimmune causes. The supravital stain showing dark inclusions attached to the membrane reflects membrane-bound hemoglobin aggregates that can occur in red cells with membrane instability and nonimmune hemolysis. The other options describe conditions with different smear features or immune-mediated destruction (positive DAT), making them less consistent with this pattern.