Should Blood Donors Be Routinely Screened for Glucose-6-Phosphate Dehydrogenase Deficiency? A Systematic Review of Clinical Studies Focusing on Patients Transfused With Glucose-6-Phosphate Dehydrogenase–Deficient Red Cells
Section snippets
Protocol
This review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines (Fig 1 and Table 1) (http://prisma-statement.org/).
Eligibility Criteria: Inclusion and Exclusion Criteria
The focus of the review is on articles that examine clinical settings involving individuals receiving whole blood or packed RBCs from healthy G6PD-deficient individuals. The studies eligible for inclusion, therefore, included randomized controlled trials, case controls, case reports, or prospective clinical series that
Level of Evidence and Data Quality
The strength of evidence reported within the 13 identified studies was then assessed using the hierarchy of evidence framework, focusing on the appropriateness and feasibility of the study, which is also reported in Table 3 [19]. The following grading was used, as proposed by Evans [19]: excellent, multicenter studies; good, randomized controlled trials, observational studies, and interpretative studies; fair, descriptive studies, before and after design, and focus groups; and poor, expert
Study Characteristics and Samples
Although the initial search identified 663 potentially relevant articles, only 13 studies met the inclusion criteria. Studies reviewed were from various countries such as Brazil (n = 1), China (n = 1), Iran (n = 2), India (n = 4), Israel (n = 2), Nigeria (n = 1), and United States (n = 2) and regions such as Americas (n = 3), Asia (n = 5), and Africa/Middle East (n = 5). Five of the research articles focused on the effects of G6PD-deficient blood transfused to adult patients, and 8 focused on the effects of
Studies Among Neonates and Children
Zekavat et al [21] undertook a clinical series study of 114 patients aged from 4 to 15 years admitted to a hospital for G6PD deficiency hemolytic anemia after broad bean ingestion. The authors examined the residual blood in transfusion blood bags, and of the 114 transfusion blood bags used across patients, 100 had G6PD-sufficient RBCs (ie, normal) and 14 had G6PD-deficient red cells. The hemoglobin level was checked after 6 hours. In both groups, hemoglobin levels rose significantly before and
Studies Among Adults
Raciti et al [29] assessed the consequences of transfusing RBC units that are G6PD deficient and contain hemoglobin variants on a 27-year-old woman with SCD. The woman attended an outpatient apheresis unit at a tertiary care medical center for scheduled RBC exchange transfusions (RBCEx) for secondary prophylaxis of stroke (blood type: O + with negative red cell antibody screen). She had been receiving RBCEx every 1.5 to 2 months as an outpatient as part of her medical treatment plan, and her last
Discussion
From the review of these 13 studies, it appears that results differed depending on the study population. The effects of G6PD-deficient transfused blood on neonates and children appear to have been more deleterious than those on adult patients. In most cases, the rise of TSB was abnormal in infants transfused with G6PD-deficient blood from 6 hours up to 60 hours after transfusion. Generally, there was also an increase in the median duration of phototherapy and a need for repeat exchange
Conclusion
Globally, most blood services do not currently consider G6PD deficiency systematically as an issue associated with sourcing blood donations. However, the potentially negative effects associated with transfused G6PD-deficient blood on recipients have received considerable comment and recommendations but, unfortunately, have been infrequently studied directly. Over a period of 40 years, only 13 studies have been identified as relevant to the subject. However, there is no conclusive evidence on
Acknowledgments
A/Prof. Andre Renzaho is supported by an ARC Future Fellowship. We would like to acknowledge Associate Professor Erica Wood, a Transfusion Medicine Specialist, and Head of the Transfusion Research Unit at the Department of Epidemiology and Preventive Medicine, for commenting on the draft.
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Thalassemia trait and G6PD deficiency in Thai blood donors
2019, Transfusion and Apheresis ScienceCitation Excerpt :Moreover, increased numbers of microparticles in G6PD-deficient patients has been reported [17]. Concerns about hemolysis and adverse outcomes in patients who were transfused with red cells from G6PD-deficient blood donors are now part of the public health debate [4,11,18]. The World Health Organization’s guidelines about blood donor selection recommend that blood from G6PD-deficient blood donors with no history of hemolysis is acceptable, but is not suitable for intrauterine transfusion, neonatal exchange transfusion, or for patients with G6PD deficiency [19].
Red cell transfusion in paediatric patients with thalassaemia and sickle cell disease: Current status, challenges and perspectives
2018, Transfusion and Apheresis ScienceCitation Excerpt :Data collected from in vitro models of transfusion [139] showed that stored RBCs from G6PD deficient donors exhibit increased haemolysis and oxidative lesions compared to control RBCs when found in transfusion-mimicking conditions, in the absence of any pathological or disease setting. According to clinical reports, transfusion of G6PD-deficient blood may have adverse effects on premature neonates, infants, recipients with G6PD deficiency themselves, and regularly transfused patients [140]. As expected, more deleterious effects have been reported on neonates and children than on adult susceptible patients.
Red blood cell components: Meeting the quantitative and qualitative transfusion needs
2016, Presse MedicaleCitation Excerpt :As such, one might expect RBCs from individuals who have damaging polymorphisms in RBC enzymes and proteins important in resisting oxidative stress would store poorly, and that these individuals would be “poor storers.” As an example, RBCs from individuals with G6PD deficiency are highly sensitive to oxidative stress; however, it remains controversial whether their refrigerator-stored RBCs are of lower quality, with suboptimal 24-hour post-transfusion recovery [25,68]. If they were, one might recommend that these individuals be excluded from the donor pool, or, at least, be identified in the donor pool [69].
Glucose 6-phosphate dehydrogenase deficient subjects may be better "storers" than donors of red blood cells
2016, Free Radical Biology and MedicineCitation Excerpt :In areas endemic for G6PD deficiency, a considerable percentage of G6PD− RBC units (>1.1% in Italy) [9] support transfusion therapy. The enrollment of G6PD deficient subjects as routine RBC donors has been questioned [3,73] and notably, the results from our in vitro model of transfusion are consistent with clinical reports. Indeed, lower 24 h post-transfusion recovery of stored RBCs from G6PD deficient donors when compared with normal RBCs was reported about fifty years ago [74].
The Preterm Infant. A High-Risk Situation for Neonatal Hyperbilirubinemia Due to Glucose-6-Phosphate Dehydrogenase Deficiency
2016, Clinics in PerinatologyCitation Excerpt :It stands to reason that the preterm, with inherent immaturity of the bilirubin conjugation system, will be at greater risk than more mature counterparts should he be transfused with G6PD-deficient blood with the potential of hemolysis. In a meta-analysis, Renzaho and colleagues59 conclude that, in general, for adult recipients, the risks of transfusion with G6PD-deficient blood are minimal. They do suggest, however, that for preterm infants or neonates requiring exchange transfusion, G6PD screening of the donor blood may be appropriate.
Eliette Husser conducted the initial search and data extraction and performed the data analysis and summary as well as drafting the first manuscript. Andre Renzaho supervised the conduct of the study. He independently verified the data extraction, provided intellectual input into the data analysis and summary and the drafting of the article, and critically reviewed the manuscript. All authors have approved its submission.