Haemolysis and methaemoglobinaemia (MetHb) are rare metabolic complications that can occur in Alkaptonuria (AKU), for which there is no curative treatment. Presented is a case of a man who had AKU, and serves as a reminder of life-threatening complications that can occur with haemolysis and MetHb. This case presents an opportunity to revisit important considerations relating to the investigation and treatment of haemolysis and MetHb with a view to raising awareness, and in doing so hopefully reducing the uniformly fatal outcome. Additionally it is proposed that treatment of haemolysis and MetHb with nitisinone is considered as a potentially lifesaving treatment as it is believed that reducing the concentration of circulating homogentisic acid will reduce oxidative stress.

溶血和高铁血红蛋白血症（MetHb）是Alkaptonuria（AKU）中可能发生的罕见代谢并发症，目前尚无治疗方法。介绍的是一名患有AKU的男人的案例，它提醒人们溶血和MetHb可能发生危及生命的并发症。此案为重新认识与溶血和MetHb的研究和治疗有关的重要考虑提供了机会，以提高认识，并希望这样做可以减少一致的致命结果。另外，由于认为降低循环中的高纯酸的浓度将减少氧化应激，因此建议用尼替尼酮治疗溶血和MetHb是可能挽救生命的治疗方法。