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The clinical impact of glucose-6-phosphate dehydrogenase deficiency in patients with sickle cell disease

Karafin, Matthew S.a; Fu, Xiaoyunb; D’Alessandro, Angeloc; Thomas, Tiffanyd; Hod, Eldad A.d; Zimring, James C.b; Field, Joshua J.a; Francis, Richard O.d

doi: 10.1097/MOH.0000000000000455

Purpose of review Glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell disease (SCD) cause hemolysis, often occurring in individuals of African descent. These disorders co-occur frequently, and possibly interact, altering clinical outcomes in SCD. However, epidemiological investigations of SCD with G6PD deficiency have produced variable results. This contribution reviews the available data about the interaction of G6PD deficiency and SCD.

Recent findings Overall, G6PD deficiency contributes few, if any, effects to laboratory values and clinical outcomes in SCD patients, but may impact transfusion efficacy. This observation is most likely because of the relatively increased G6PD activity in the young red blood cell (RBC) population seen in SCD patients with or without G6PD deficiency. In addition, G6PD deficiency possibly interacts with other genetic modifiers, such as α thalassemia, hemoglobin F levels and SCD haplotype.

Summary Although G6PD deficiency is relatively common, it does not appear to clinically impact patients with SCD. Nonetheless, it is important to evaluate G6PD status in patients with SCD to avoid the use of medications that may cause hemolysis. Future studies evaluating the clinical impact of transfusions from G6PD-deficient RBC donors would be of the greatest benefit to the current literature.

aMedical Sciences Institute, BloodCenter of Wisconsin, Milwaukee, Wisconsin

bBloodWorks Northwest, Seattle, Washington

cDepartment of Biochemistry and Molecular Genetics, University of Colorado, Denver, Colorado

dLaboratory of Transfusion Biology, Department of Pathology and Cell Biology, Columbia University Medical Center-New York Presbyterian Hospital, New York, New York, USA

Correspondence to Richard O. Francis, MD, PhD, Laboratory of Transfusion Biology, Department of Pathology and Cell Biology, Columbia University College of Physicians and Surgeons, P&S Building, Room 14–434, 630 West 168th Street, New York, NY 10032, USA. Tel: +1 212 342 4569; fax: +1 212 305 4489; e-mail:

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