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多发性骨髓瘤中的高氨血症性脑病:一例报告

Hyperammonemic Encephalopathy in Multiple Myeloma: A Case Report.

作者信息

Vahora Ilma, Panneerselvam Ezhil, Deoker Abhizith

机构信息

Internal Medicine, Texas Tech University Health Sciences Center El Paso, El Paso, USA.

Internal Medicine, Texas Tech University Health Sciences Center El Paso Paul L. Foster School of Medicine, El Paso, USA.

出版信息

Cureus. 2023 Jan 10;15(1):e33626. doi: 10.7759/cureus.33626. eCollection 2023 Jan.

Abstract

Multiple myeloma (MM) typically presents as lytic bony lesions, hypercalcemia, anemia, and renal failure. Only a few cases of hyperammonemic encephalopathy (HE) attributed to multiple myeloma have been reported. We report a case of a 68-year-old Hispanic female diagnosed with multiple myeloma and presented with altered mental status and elevated ammonia levels found to have HE. The pathology behind HE is associated with higher ammonia levels produced by myeloma cell lines in the absence of liver disease. Due to the wide range of differentials for altered mental status (AMS), HE often gets missed and causes delayed treatment and the associated higher mortality. The primary treatment is chemotherapy. Lactulose and rifaximin must be initiated; however, it is ineffective if solely used. In our case, chemotherapy was not considered a treatment option in light of the patient's pancytopenia and infection. Our case is unique, as despite adequately treating other commonly suspected causes of AMS such as infection, there was no expected improvement in the patient's clinical status noticed, eventually leading to intubation due to worsening AMS. Given the patient's history of multiple myeloma, non-compliance with chemotherapy before presentation, and elevated ammonia levels raised suspicion for HE. Clinicians are encouraged to acquaint themselves with HE as a differential for patients presenting with MM flare and AMS, specifically when other potential causes of AMS are ruled out and addressed.

摘要

多发性骨髓瘤(MM)通常表现为溶骨性骨病变、高钙血症、贫血和肾衰竭。仅有少数几例归因于多发性骨髓瘤的高氨血症性脑病(HE)的病例报告。我们报告一例68岁西班牙裔女性,被诊断为多发性骨髓瘤,出现精神状态改变和血氨水平升高,确诊为HE。HE背后的病理与骨髓瘤细胞系在无肝脏疾病情况下产生的较高血氨水平相关。由于精神状态改变(AMS)的鉴别诊断范围广泛,HE常常被漏诊,导致治疗延迟和相关的较高死亡率。主要治疗方法是化疗。必须开始使用乳果糖和利福昔明;然而,单独使用时无效。在我们的病例中,鉴于患者全血细胞减少和感染,化疗未被视为一种治疗选择。我们的病例很独特,因为尽管对其他常见的疑似AMS病因如感染进行了充分治疗,但患者的临床状况并未出现预期改善,最终由于AMS恶化导致插管。鉴于患者有多发性骨髓瘤病史、就诊前未遵行化疗以及血氨水平升高,引发了对HE的怀疑。鼓励临床医生了解HE,将其作为MM病情发作和AMS患者的鉴别诊断,特别是在排除并处理了AMS的其他潜在病因时。

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本文引用的文献

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[Hyperammonemic encephalopathy as the presenting feature of multiple myeloma].[高氨血症性脑病作为多发性骨髓瘤的首发表现]
Med Clin (Barc). 2016 Nov 18;147(10):472-473. doi: 10.1016/j.medcli.2016.07.004. Epub 2016 Aug 24.
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Hyperammonemic encephalopathy in multiple myeloma.多发性骨髓瘤中的高氨血症性脑病
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