Arun V J, Deorukhkar Anuradha, Rafi Aboobacker Mohamed, Charles Deepak, Devendra Rati, Innah Susheela J, Kedar Prabhakar
Department of Transfusion Medicine and Clinical Lab, Jubilee Mission Medical College and Research Institute, Thrissur, Kerala, India.
Department of Haematogenetics, ICMR-National Institute of Immunohematology, King Edward Memorial Hospital Campus, Parel, Mumbai, India.
Asian J Transfus Sci. 2022 Jan-Jun;16(1):128-131. doi: 10.4103/ajts.ajts_152_20. Epub 2022 May 26.
A 27-year-old female patient who came for branchial cyst excision was found to have cyanosis and a saturation gap during preanesthetic check-up and hence she was referred to haematology for further workup. She had a Hb of 9 gm% with all other baseline tests as normal. Blood samples were sent for methaemoglobin estimation and related work up to the National Institute of Immunohematology (NIIH) Mumbai. She was diagnosed as a case of Methemoglobinemia with a methaemoglobin level of 68.7% with NADH cytochrome B5 reductase activity of 10.82 IU/g Hb. The drug of choice for treatment is Methylene blue and hence G6PD deficiency had to be ruled out prior to initiating therapy. She was found to have a concurrent existence of G6PD deficiency. The blood sample was further sent to NIIH for genetic confirmation. We avoided methylene blue and other precipitating factors that could trigger a haemolysis. She was further consulted by the Patient blood management team to optimize her erythropoiesis and avoid unnecessary transfusions. Anaesthetic consultation and planning were done to avoid drugs that could induce haemolysis. She was started on Vitamin C, Niacin, hematinic and advised to follow up after a month. She was symptomatically better. Cyanosis had reduced, and Hb improved to 12 gm%. She was taken up for surgery with all precautions. The surgery and the post-operative period were uneventful. She was discharged on postoperative day 4 with an advice to continue Vitamin C & Niacin and to follow-up in Haematology OPD after a month.
一名前来进行鳃裂囊肿切除术的27岁女性患者在麻醉前检查时发现有发绀和氧饱和度差值，因此被转至血液科做进一步检查。她的血红蛋白水平为9克%，其他所有基线检查均正常。血液样本被送往孟买国家免疫血液学研究所（NIIH）进行高铁血红蛋白测定及相关检查。她被诊断为高铁血红蛋白血症，高铁血红蛋白水平为68.7%，烟酰胺腺嘌呤二核苷酸（NADH）细胞色素B5还原酶活性为10.82国际单位/克血红蛋白。治疗的首选药物是亚甲蓝，因此在开始治疗前必须排除葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症。结果发现她同时存在G6PD缺乏症。血液样本被进一步送往NIIH进行基因确认。我们避免使用亚甲蓝和其他可能引发溶血的诱发因素。患者血液管理团队进一步会诊，以优化她的红细胞生成并避免不必要的输血。进行了麻醉会诊和规划，以避免使用可能诱发溶血的药物。她开始服用维生素C、烟酸、补血剂，并被告知一个月后复诊。她的症状有所改善。发绀减轻，血红蛋白水平提高到12克%。她在采取了所有预防措施后接受了手术。手术及术后过程顺利。她于术后第4天出院，医嘱继续服用维生素C和烟酸，并在一个月后到血液科门诊复诊。
