To test the hypothesis that transfusion of blood donated by individuals with glucose-6-phosphate dehydrogenease (G6PD) deficiency may result in a hemolytic reaction, we conducted a prospective longitudinal study in which 10 patients transfused with 1 unit of G6PD-deficient and 1 unit of normal red blood cells (RBC) were compared with 10 patients transfused with 2 units of age-matched normal RBC. We found that 24h after transfusion serum bilirubin (µmol/l) in the recipients of G6PD-deficient RBC was significantly higher than in the recipients of normal RBC (36±14 vs. 18±5, respectively, p>0.004). A parallel increase was found in the serum lactate dehydrogenase (LDH; IU/l) between the two groups (378±151 vs. 264±56, p<0.001). The difference in serum bilirubin (26±10 vs. 15±5, p<0.03) was still noted 48 h after transfusion, with only a marginal difference (p<0.08) in LDH. We conclude that an immediate posttransfusional hemolytic reaction can occur in recipients of G6PD- deficient RBC and therefore suggest that the differential diagnosis of posttransfusional hemolysis, particularly in populations where G6PD deficiency is prevalent, includes transfusion of erythrocytes from G6PD-deficient donors.

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