背景:Favism是众所周知的急性溶血性贫血的病因。很少,由于葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症患者摄入蚕豆,也可能发生高铁血红蛋白血症。文献中很少报道这种共现的病例。
方法:我们报告一例47岁患者,在食用蚕豆2天后出现黄疸。
方法:实验室检查显示贫血有溶血的证据(高网织红细胞计数,高间接胆红素,外周涂片中的叮咬细胞)。血气显示高高铁血红蛋白水平。G6PD酶水平降低证实了G6PD缺乏的诊断。
方法:患者持续补充氧气。建议他避免食用可能导致急性溶血的食物和药物。
结果:血氧饱和度逐渐提高。2天后患者出院,无任何并发症。
结论:G6PD缺乏症患者可发生急性溶血性贫血和食用蚕豆继发的高铁血红蛋白血症。这些患者不应接受亚甲蓝治疗,以免溶血恶化。
BACKGROUND: Favism is a well-known cause of acute hemolytic anemia. Rarely, methemoglobinemia can also happen because of fava bean ingestion in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency. Few cases with this co-occurrence have been reported in the literature.
METHODS: We report a
case of a 47-year-old patient who presented with jaundice that started 2 days after eating fava beans.
METHODS: Laboratory investigations revealed anemia with evidence of hemolysis (high reticulocytes count, high indirect bilirubin, bite cells in peripheral smear). Blood gases showed high methemoglobin level. Reduced level of G6PD enzyme confirmed the diagnosis of G6PD deficiency.
METHODS: The patient was kept on supplemental oxygen. He was counselled to avoid food and drugs that can cause acute hemolysis.
RESULTS: Oxygen saturation improved gradually. The patient was discharged without any complications after 2 days.
CONCLUSIONS: Patients with G6PD deficiency can develop both acute hemolytic anemia and methemoglobinemia secondary to fava beans ingestion. These patients should not receive methylene blue to avoid worsening hemolysis.