氨砜致G6PD酶缺乏性溶血性贫血1例

Ali Doğan, Narin Yıldırım Doğan, Ömer Ekinci, Sinan Demircioğlu, C. Demir, Cihan Ural, R. Esen
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引用次数: 0

摘要

溶血性贫血的特点是由于红细胞溶血增加而导致循环红细胞数量减少。葡萄糖-6-磷酸脱氢酶(G6PD)缺乏是与溶血有关的最常见的红细胞酶缺陷。G6PD酶通过参与谷胱甘肽代谢,保护红细胞免受氧化应激,从而消除红细胞的溶血。G6PD酶缺乏症相关的溶血性贫血可能表现为新生儿黄疸或由于暴露于感染,寄生虫病和药物治疗而变得明显。氨苯砜是医生在治疗寻常性天疱疮等许多皮肤病时首选的药物,在G6PD酶缺乏的情况下会导致溶血。在这种非免疫性溶血中,接触球蛋白低,库姆斯试验呈阴性。溶血性贫血是使用氨苯砜后可能出现的严重并发症,可通过在氨苯砜治疗前检测G6PD酶水平来预防。在这种情况下,我们强调使用氨砜患者的溶血性贫血可能是由于G6PD酶缺乏。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A case of dapsone-induced hemolytic anemia related to G6PD enzyme deficiency
Hemolytic anemia is characterized by a decrease in the number of circulating erythrocytes due to an increase in their hemolysis. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common erythrocyte enzyme defects related to hemolysis. The G6PD enzyme abrogates the hemolysis of erythrocytes by protecting them against oxidative stress due to its involvement in the glutathione metabolism. G6PD enzyme deficiency-related hemolytic anemia may present as neonatal jaundice or become manifest due to exposure to infections, favism and medications later in life. Dapsone is a medication that is preferred by doctors in the treatment of many dermatological disorders such as pemphigus vulgaris, and leads to hemolysis in the presence of G6PD enzyme deficiency. In this type of non-immune hemolysis, haptoglobulin is low and Coombs' tests are negative. Hemolytic anemia, a serious complication that may appear subsequent to dapsone use, can be prevented by testing G6PD enzyme levels prior to dapsone therapy. In this case, we emphasized that the hemolytic anemia in the patient using dapsone may be due to G6PD enzyme deficiency.
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