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患者患有 G6PD 缺乏症,吸入一氧化氮后发生合并氧化溶血和高铁血红蛋白血症。

Concomitant oxidative haemolysis and methaemoglobinaemia following inhaled nitric oxide in a patient with G6PD deficiency.

机构信息

Department of Medicine, The Johns Hopkins Hospital, Baltimore, Maryland, USA.

Division of Hematology, The Johns Hopkins Hospital, Baltimore, Maryland, USA.

出版信息

BMJ Case Rep. 2024 Aug 29;17(8):e259709. doi: 10.1136/bcr-2024-259709.

DOI:10.1136/bcr-2024-259709
PMID:39209743
Abstract

We report the case of a man in his 50s who developed acute respiratory distress syndrome and right heart failure, necessitating intubation and initiation of inhaled nitric oxide (iNO) to decrease right ventricular afterload and improve the right heart function. The course was complicated by acute anaemia, with a diagnostic workup revealing methaemoglobinaemia and evidence of oxidative haemolysis indicated by blister and bite cells on peripheral blood film. The patient received conservative management, including successive red blood cell transfusion and gradual iNO weaning due to suspected glucose-6-phosphate dehydrogenase (G6PD) deficiency. Discontinuation of iNO led to the resolution of both oxidative haemolysis and methaemoglobinaemia. Subsequent enzymatic assay, conducted 4 months later, confirmed G6PD deficiency. This case highlights a rare instance of concurrent methaemoglobinaemia and oxidative haemolytic anaemia following iNO in a patient with underlying G6PD deficiency.

摘要

我们报告了一例 50 多岁男性患者,他患有急性呼吸窘迫综合征和右心衰竭,需要插管和使用吸入一氧化氮(iNO)来降低右心室后负荷并改善右心功能。该患者病情复杂,出现急性贫血,诊断检查显示高铁血红蛋白血症,并在外周血涂片上发现了水疱和咬细胞,提示有氧化性溶血。患者接受了保守治疗,包括连续输血和逐步减少 iNO 的使用,因为怀疑存在葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症。停止使用 iNO 后,氧化性溶血和高铁血红蛋白血症均得到缓解。随后在 4 个月后进行的酶学检测证实了 G6PD 缺乏症。本例罕见地提示了在存在潜在 G6PD 缺乏症的患者中,iNO 治疗后会同时发生高铁血红蛋白血症和氧化性溶血性贫血。

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