Bone Marrow Failure Disorders

In their review and analysis, the authors propose a model for disturbed red blood cell maturation in pyruvate kinase deficiency (PKD) in which glutathione depletion leads to relative GPX4 deficiency that disrupts mitophagy. The resulting failure of reticulocyte maturation, the authors note, can be defined as late-stage ineffective erythropoiesis. They conclude that their hypothesis “provides a starting point for future research into new therapeutic possibilities, which have the ability to correct the oxidative imbalance due to lack of GPX4” in patients with PKD.