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一名葡萄糖-6-磷酸脱氢酶(G6PD)缺乏的男性出现严重急性溶血性贫血并伴有严重高铁血红蛋白血症。

Severe acute haemolytic anaemia associated with severe methaemoglobinaemia in a G6PD-deficient man.

作者信息

Rehman Abdul, Shehadeh Mohanad, Khirfan Diala, Jones Akhnuwhkh

机构信息

Department of Biological and Biomedical Sciences, Aga Khan University, Karachi, Pakistan.

Department of Medicine, Hamad Medical Corporation, Doha, Qatar.

出版信息

BMJ Case Rep. 2018 Mar 28;2018:bcr-2017-223369. doi: 10.1136/bcr-2017-223369.

Abstract

Methaemoglobin is a form of haemoglobin in which the ferrous (Fe) ion contained in the iron-porphyrin complex of haem is oxidised to its ferric (Fe) state. Methaemoglobinaemia, the presence of methaemoglobin in the blood, is most commonly treated with methylene blue. However, methylene blue cannot be used in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency as it is ineffective in such patients and it can worsen G6PD deficiency haemolysis. We report the case of a 30-year-old man who presented with clinical features of G6PD deficiency-associated haemolysis and was found to have severe methaemoglobinaemia (35%). He was administered blood transfusions and intravenous ascorbic acid. His methaemoglobinaemia resolved within 24 hours. This case demonstrates the successful management of a patient with severe methaemoglobinaemia in the setting of G6PD deficiency haemolysis. Emergency physicians should be aware of the possible co-occurrence of severe methaemoglobinaemia in a patient with G6PD deficiency haemolysis.

摘要

高铁血红蛋白是血红蛋白的一种形式,其中血红素的铁-卟啉复合物中所含的亚铁(Fe)离子被氧化为其三价铁(Fe)状态。高铁血红蛋白血症,即血液中存在高铁血红蛋白,最常用亚甲蓝治疗。然而,亚甲蓝不能用于葡萄糖-6-磷酸脱氢酶(G6PD)缺乏的患者,因为它对此类患者无效,且会加重G6PD缺乏性溶血。我们报告一例30岁男性病例,该患者表现出G6PD缺乏相关溶血的临床特征,且被发现患有严重高铁血红蛋白血症(35%)。给予其输血和静脉注射维生素C。其高铁血红蛋白血症在24小时内消退。该病例证明了在G6PD缺乏性溶血情况下成功治疗严重高铁血红蛋白血症患者的方法。急诊医生应意识到G6PD缺乏性溶血患者可能同时出现严重高铁血红蛋白血症。

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本文引用的文献

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