Medical Sciences Institute, BloodCenter of Wisconsin, Milwaukee, Wisconsin.
BloodWorks Northwest, Seattle, Washington.
Curr Opin Hematol. 2018 Nov;25(6):494-499. doi: 10.1097/MOH.0000000000000455.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell disease (SCD) cause hemolysis, often occurring in individuals of African descent. These disorders co-occur frequently, and possibly interact, altering clinical outcomes in SCD. However, epidemiological investigations of SCD with G6PD deficiency have produced variable results. This contribution reviews the available data about the interaction of G6PD deficiency and SCD.
Overall, G6PD deficiency contributes few, if any, effects to laboratory values and clinical outcomes in SCD patients, but may impact transfusion efficacy. This observation is most likely because of the relatively increased G6PD activity in the young red blood cell (RBC) population seen in SCD patients with or without G6PD deficiency. In addition, G6PD deficiency possibly interacts with other genetic modifiers, such as α thalassemia, hemoglobin F levels and SCD haplotype.
Although G6PD deficiency is relatively common, it does not appear to clinically impact patients with SCD. Nonetheless, it is important to evaluate G6PD status in patients with SCD to avoid the use of medications that may cause hemolysis. Future studies evaluating the clinical impact of transfusions from G6PD-deficient RBC donors would be of the greatest benefit to the current literature.
葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症和镰状细胞病（SCD）可引起溶血性贫血，常发生于非裔人群。这两种疾病常同时发生，并可能相互作用，从而改变 SCD 的临床结局。然而，对伴或不伴 G6PD 缺乏症的 SCD 进行的流行病学调查得出了不同的结果。本综述回顾了有关 G6PD 缺乏症与 SCD 相互作用的现有数据。
总体而言，G6PD 缺乏症对 SCD 患者的实验室值和临床结局几乎没有影响，但可能会影响输血效果。这种观察结果最有可能是因为 SCD 患者的年轻红细胞（RBC）群体中 G6PD 活性相对增加，无论是否存在 G6PD 缺乏症。此外，G6PD 缺乏症可能与其他遗传修饰因子相互作用，如α地中海贫血、血红蛋白 F 水平和 SCD 单倍型。
尽管 G6PD 缺乏症较为常见，但似乎不会对 SCD 患者的临床状况产生影响。尽管如此，在 SCD 患者中评估 G6PD 状态以避免使用可能引起溶血的药物仍然很重要。评估来自 G6PD 缺乏 RBC 供体的输血对当前文献具有最大临床意义的未来研究将大有裨益。
