葡萄糖-6-磷酸脱氢酶缺乏症在镰状细胞病中 Duffy 阴性红细胞输注中更为常见。

Glucose-6-phosphate dehydrogenase deficiency is more prevalent in Duffy-null red blood cell transfusion in sickle cell disease.

机构信息

Center for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA.

Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.

出版信息

Transfusion. 2022 Mar;62(3):551-555. doi: 10.1111/trf.16806. Epub 2022 Jan 19.

DOI:10.1111/trf.16806

PMID:35044697

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8940658/

Abstract

BACKGROUND

Resistance to malaria infection may be conferred by erythrocyte genetic variations including glucose-6-phosphate dehydrogenase (G6PD) deficiency and lack of Duffy antigens. In red blood cell (RBC) transfusion, G6PD deficiency may shorten transfusion survival. Because Duffy-null units are commonly transfused in sickle cell disease (SCD) due to antigen matching protocols, we examined whether Duffy-null donor RBC units have a higher prevalence of G6PD deficiency.

MATERIALS AND METHODS

Pediatric patients with SCD on chronic transfusion therapy were followed prospectively for multiple transfusions. RBC unit segments were collected to measure G6PD activity and RBC genotyping. The decline in donor hemoglobin (ΔHbA) following transfusion was assessed from immediate posttransfusion estimates and HbA measurements approximately 1 month later.

RESULTS

Of 564 evaluable RBC units, 59 (10.5%) were G6PD deficient (23 severe, 36 moderate deficiency); 202 (37.6%) units were Duffy-null. G6PD deficiency occurred in 40 (19.8%) Duffy-null units versus 15 (4.5%) Duffy-positive units (p < .0001). In univariate analysis, the fraction of Duffy-null RBC units per transfusion was associated with greater decline in HbA (p = .038); however, in multivariate analysis, severe G6PD deficiency (p = .0238) but not Duffy-null RBC (p = .0139) were associated with ΔHbA.

CONCLUSION

Selection of Duffy-null RBC units may result in shorter in vivo survival of transfused RBCs due to a higher likelihood of transfusing units from G6PD deficient donors.

摘要

背景

对疟疾感染的抵抗力可能由红细胞遗传变异引起，包括葡萄糖-6-磷酸脱氢酶（G6PD）缺乏和缺乏 Duffy 抗原。在红细胞（RBC）输血中，G6PD 缺乏可能会缩短输血的存活时间。由于抗原匹配方案，Duffy 阴性单位通常在镰状细胞病（SCD）中输注，因此我们检查了 Duffy 阴性供体 RBC 单位是否更普遍存在 G6PD 缺乏。

材料和方法

接受慢性输血治疗的 SCD 儿科患者进行了前瞻性随访，以进行多次输血。收集 RBC 单位段以测量 G6PD 活性和 RBC 基因分型。从输血后的即时估计和大约 1 个月后的 HbA 测量评估供体血红蛋白（ΔHbA）的下降。

结果

在 564 个可评估的 RBC 单位中，有 59 个（10.5%）为 G6PD 缺乏症（23 个严重，36 个中度缺乏症）；202 个（37.6%）单位为 Duffy 阴性。在 Duffy 阴性单位中发生了 40 个（19.8%）G6PD 缺乏症，而在 Duffy 阳性单位中发生了 15 个（4.5%）（p<0.0001）。在单变量分析中，每输血的 Duffy 阴性 RBC 单位分数与 HbA 下降幅度更大相关（p=0.038）；然而，在多变量分析中，严重的 G6PD 缺乏症（p=0.0238）而非 Duffy 阴性 RBC（p=0.0139）与ΔHbA 相关。