Observational studyAmerican journal of hematology2018
Glucose-6-phosphate-dehydrogenase deficient red blood cell units are associated with decreased posttransfusion red blood cell survival in children with sickle cell disease.
Observational study in American journal of hematology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
What it found
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
26 citing papers in PubMed, 40 citations in OpenAlex.
- Red blood cell transfusion survival in sickle cell disease is reduced by donor characteristics and recipient spleen activity.Blood advances · 2026Trial
- Donor glucose-6-phosphate dehydrogenase deficiency decreases blood quality for transfusion.The Journal of clinical investigation · 2020Trial
- NO modulates human airway smooth muscle function by altering glucose-6-phosphate dehydrogenase effects on sGC function in asthma.Redox biology · 2026Article
- Blood Transfusion and ABO Blood Groups in Sickle Cell Disease.Medicina (Kaunas, Lithuania) · 2026Article
- Short- and long-term effects of transfusion in β-thalassemia: a longitudinal study of transfusion efficiency factors.Blood advances · 2026Article
- HypomorphicHemaSphere · 2026Article
- Survival of transfused red blood cells from a donor with alpha-thalassemia trait in a recipient with sickle cell disease.Transfusion · 2024Observational
- Post-transfusion biotin-labeled red blood cell survival studies in pediatric sickle cell disease with antibodies of uncertain significance.Transfusion · 2024Article
- Article
- Alloimmunization and hyperhemolysis in sickle cell disease.Hematology. American Society of Hematology. Education Program · 2023Article
- Acquired Glucose-6-Phosphate Dehydrogenase Deficiency.Journal of clinical medicine · 2022Review
- Observational
- Glucose-6-phosphate dehydrogenase deficiency is more prevalent in Duffy-null red blood cell transfusion in sickle cell disease.Transfusion · 2022Article
- Donor genetic and nongenetic factors affecting red blood cell transfusion effectiveness.JCI insight · 2022Article
- Red cell proteasome modulation by storage, redox metabolism and transfusion.Blood transfusion = Trasfusione del sangue · 2022Article
- Donor sex, age and ethnicity impact stored red blood cell antioxidant metabolism through mechanisms in part explained by glucose 6-phosphate dehydrogenase levels and activity.Haematologica · 2021Article
- Current Understanding of the Relationship between Blood Donor Variability and Blood Component Quality.International journal of molecular sciences · 2021Review
- How Do Red Blood Cells Die?Frontiers in physiology · 2021Review
- ABO blood group should be considered and reported when red blood cell exchange transfusion is used to treat Plasmodiumfalciparum Malaria patients.Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine · 2020Article
- Nicotine exposure increases markers of oxidant stress in stored red blood cells from healthy donor volunteers.Transfusion · 2020Article
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 1 country.
Funding
Abstract
Chronic transfusion therapy (CTT) for sickle cell disease (SCD) reduces disease morbidity by suppressing the amount of circulating hemoglobin S (HbS)-containing red blood cells (RBC). The effectiveness of CTT depends on the rate of RBC clearance. Glucose-6-phosphate dehydrogenase (G6PD) deficient donor RBC may exhibit increased hemolysis, but it is unknown if transfusion of these units results in less effective transfusion outcomes in SCD. Children with SCD on CTT were followed prospectively for multiple transfusions. G6PD activity of transfused units was measured prior to expiration date. HbA clearance (ΔHbA) was calculated as the difference of estimated posttransfusion HbA to the pretransfusion HbA of the subsequent transfusion episode. Sixty-two patients received 388 transfusions. Of 755 RBC units, 687 (91%) had normal G6PD (>60% activity), 38 (5%) had moderately low G6PD (10-60% activity), and 30 (4%) had severely low G6PD (<10% activity). Of 358 evaluable transfusions, 54 (15%) included ≥1 G6PD deficient units, and 22 (6%) had ≥1 severely deficient units. The proportion of the transfusion episode consisting of G6PD deficient units was associated with increased ΔHbA for all G6PD deficient units (P = .05) and for severely G6PD deficient units (P = .0070). In multivariate mixed effects modeling, ΔHbA was positively associated with severely G6PD deficient units (P = .0074) and RBC alloimmunization (P = .03) and negatively associated with recipient splenectomy (P = .015). Higher ΔHbA was associated with higher HbS and reticulocyte counts at the subsequent transfusion episode. In conclusion, G6PD deficient RBC transfusions may have shorter in vivo survival and adversely affect the suppression of sickle erythropoiesis.
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