Khan Maliha, Paul Shilpa, Farooq Saad, Oo Thein Hlaing, Ramshesh Priya, Jain Nitin
Department of Leukemia, MD Anderson Cancer Center, 1515 Holcombe Blvd., Unit 428, Houston, TX 77030,. United States.
Curr Drug Saf. 2017;12(1):13-18. doi: 10.2174/1574886312666170111151246.
Rasburicase is commonly used in patients with hematologic malignancies for tumor lysis syndrome prophylaxis and management. Methemoglobinemia is a serious rare adverse effect of rasburicase, more common in patients with G6PD deficiency. Prompt diagnosis and appropriate management of this condition can make the difference between successful recovery and significant morbidity. Here we discuss the link of rasburicase with methemoglobinemia and the pathophysiology behind increased incidence of this side effect in G6PD deficient patients.
We report the case of a 73-year-old African American man who developed methemoglobinemia on rasburicase treatment, who was later confirmed to be G6PD deficient. We reviewed the literature using Pubmed and Google Scholar using the following key words: "methemoglobinemia", "rasburicase", "urate oxidase", tumor lysis syndrome", G6PD deficiency", "hemolytic anemia" and "hyperuricemia".
Rasburicase-induced methemoglobinemia is more common in patients with G6PD deficiency, and rasburicase is therefore contraindicated in these patients. Clinical presentation includes cyanosis, pallor, methemoglobin levels of 8-12%, and oxygen saturation gap which is evident from ABG analysis, though pulse oximetry is normal. Treatment consists of oxygen supplementation, ascorbic acid and blood transfusion. Importantly, methylene blue is avoided as therapy in G6PD deficiency as it can worsen the methemoglobinemia.
Rasburicase-induced methemoglobinemia is a serious concern, especially in African- American patients. It should be considered when clinical signs and symptoms are present. Knowledge of this side effect is important in early diagnosis and successful management of the condition.
拉布立酶常用于血液系统恶性肿瘤患者以预防和治疗肿瘤溶解综合征。高铁血红蛋白血症是拉布立酶一种严重的罕见不良反应，在葡萄糖-6-磷酸脱氢酶（G6PD）缺乏的患者中更常见。对这种情况的及时诊断和恰当处理可能决定能否成功康复或出现严重并发症。在此，我们讨论拉布立酶与高铁血红蛋白血症的关联以及G6PD缺乏患者中这种副作用发生率增加背后的病理生理学机制。
我们报告了一例73岁非裔美国男性在接受拉布立酶治疗时发生高铁血红蛋白血症的病例，该患者后来被证实存在G6PD缺乏。我们使用关键词“高铁血红蛋白血症”“拉布立酶”“尿酸氧化酶”“肿瘤溶解综合征”“G6PD缺乏”“溶血性贫血”和“高尿酸血症”在PubMed和谷歌学术上检索文献。
拉布立酶诱发的高铁血红蛋白血症在G6PD缺乏患者中更常见，因此这些患者禁用拉布立酶。临床表现包括发绀、面色苍白、高铁血红蛋白水平达8% - 12%，以及通过动脉血气分析可明显看出的氧饱和度差值，尽管脉搏血氧饱和度正常。治疗包括吸氧、补充维生素C和输血。重要的是，G6PD缺乏患者应避免使用亚甲蓝治疗，因为它会加重高铁血红蛋白血症。
拉布立酶诱发的高铁血红蛋白血症是一个严重问题，尤其是在非裔美国患者中。出现临床症状和体征时应予以考虑。了解这种副作用对于早期诊断和成功处理该病症很重要。
