Glucose-6-phosphate dehydrogenase deficiency is more prevalent in Duffy-null red blood cell transfusion in sickle cell disease.
Glucose-6-phosphate dehydrogenase deficiency is more prevalent in Duffy-null red blood cell transfusion in sickle cell disease.
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DOI：
10.1111/trf.16806
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发表时间：
2022-03
期刊：
影响因子：
2.9
通讯作者：
Fasano RM
中科院分区：
文献类型：
--
作者：
Yee ME;Francis RO;Luban NLC;Easley KA;Lough CM;Roback JD;Josephson CD;Fasano RM
对疟疾感染的抵抗力可能由红细胞遗传变异赋予，包括葡萄糖-6-磷酸脱氢酶（G6 PD）缺乏和缺乏达菲抗原。在输注红细胞（RBC）时，G6 PD缺乏可缩短输血存活期。由于抗原匹配协议，在镰状细胞病（SCD）中通常输注Duffy无效单位，因此我们检查了Duffy无效供体RBC单位是否具有更高的G6 PD缺乏症患病率。前瞻性随访接受长期输血治疗的SCD儿童患者的多次输血。收集RBC单位片段以测量G6 PD活性和RBC基因分型。根据输血后立即估计值和大约1个月后的HbA测量值评估输血后供体血红蛋白（ΔHbA）的下降。在564个可评价的RBC单位中，59个（10.5%）为G6 PD缺乏（23个重度，36个中度缺乏）; 202个（37.6%）单位为Duffy无效。G6 PD缺陷发生在40个（19.8%）Duffy无效单位和15个（4.5%）Duffy阳性单位中（p<0.0001）。在单变量分析中，每次输血的Duffy-null RBC单位分数与HbA的更大下降相关（p=0.038）;然而，在多变量分析中，重度G6 PD缺乏（p=0.0238）而非Duffy-null RBC（p=0.0139）与ΔHbA相关。选择Duffy无效RBC单位可能导致输注RBC的体内存活期较短，这是由于使用来自G6 PD缺陷供体的单位的可能性较高。
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. 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. Decline in donor hemoglobin (ΔHbA) following transfusion was assessed from immediate post-transfusion estimates and HbA measurements approximately 1 month later. 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<0.0001). In univariate analysis, the fraction of Duffy-null RBC units per transfusion was associated with greater decline in HbA (p=0.038); however, in multivariate analysis, severe G6PD deficiency (p=0.0238) but not Duffy-null RBC (p=0.0139) were associated with ΔHbA. 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.
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影响因子：
2.9
作者：
Francis, Richard O.;Jhang, Jeffrey;Spitalnik, Steven L.
通讯作者：
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