Blood Transfusion - 4 2019 (July-August)

Impact of G6PD status on red cell storage and transfusion outcomes


Key words: glucosephosphate dehydrogenase deficiency, blood transfusion, haemolysis, metabolomics, sickle cell
Publication Date: 2019-07-04


There are inter-individual differences in the quality of refrigerator-stored red blood cells (RBCs). Possible sources of these variations include nutritional and genetic factors. Glucose-6-phosphate dehydrogenase (G6PD) deficiency, the most common enzyme deficiency worldwide that affects the ability of RBCs to respond to oxidative stress, has been implicated as a genetic factor that affects the quality of stored RBCs. This review considers the literature concerning G6PD-deficient RBCs. It discusses RBC unit variables such as in vitro storage, 24-hour post-transfusion recovery (PTR), post-transfusion survival, and post-transfusion clinical outcomes.
There are several differences in the in vitro storage characteristics between G6PD-deficient and G6PD-normal RBCs. Recent studies identified differences in the pathways related to glycolysis, purine metabolism, glutathione homeostasis, and fatty acid metabolism. In vitro experiments modelling the transfusion of G6PD-deficient RBCs, as well as autologous PTR studies in vivo, demonstrate increased haemolysis and decreased PTR, respectively, both indicators of a decrease in quality as compared to G6PD-normal RBCs. Finally, studies transfusing G6PD-deficient and G6PD-normal RBCs show that, in certain clinical settings, G6PD-deficient RBCs are associated with increased haemolysis.
In summary, G6PD deficiency is associated with a decrease in the quality of RBCs after storage and its impact is often under-estimated. Understanding the underlying mechanisms by which G6PD deficiency affects RBC storage and transfusion outcomes may provide important clues to help optimise the future efficacy and safety of transfusions.



Matthew S. Karafin - Medical Sciences Institute, BloodCenter of Wisconsin, Milwaukee, WI

Richard O. Francis - Laboratory of Transfusion Biology, Department of Pathology and Cell Biology, Columbia University Irving Medical Center-New York Presbyterian Hospital, New York, NY, United States of America

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