Review

Blood Transfusion - 4 2019 (July-August)

Impact of G6PD status on red cell storage and transfusion outcomes

Authors

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

Abstract

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.

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Authors

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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