葡萄糖—6—磷酸脱氢酶(G6PD)缺乏症是一种主要表现溶血的遗传性代谢疾患,遗传方式为X连锁不完全显性遗传,具有不同的表现度。患者的G6PD活性或稳定性显著降低,溶血性贫血一般呈间歇性发作,但某些变异型可有慢性自发性溶血的表现。在G6PD众多的变异型中,有不少在服用蚕豆或伯氨喹啉类药物后,会诱发急性溶血。 Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a hereditary metabolic disorder that predominantly manifests as hemolysis and is inherited in an X-linked incompletely dominant inheritance with varying degrees of performance. Patients with G6PD activity or stability was significantly reduced, hemolytic anemia usually intermittent episodes, but some variants may have chronic spontaneous hemolysis. Among the many variants of G6PD, many take acute haemolysis after taking fava beans or primaquine drugs.