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高氨血症性脑病:多发性骨髓瘤的一种独特表现。

Hyperammonemic Encephalopathy: A Unique Presentation of Multiple Myeloma.

作者信息

Douedi Steven, Kapadia Shruti, AlAzzawi Mohammed, Sen Shuvendu

机构信息

Internal Medicine, Jersey Shore University Medical Center, Neptune, USA.

出版信息

Cureus. 2021 Jan 19;13(1):e12781. doi: 10.7759/cureus.12781.

Abstract

Multiple myeloma is a malignancy of plasma cells which are commonly found in the bone marrow. Known for causing a wide range of symptoms and affecting various organ systems, multiple myeloma is a rare malignancy with the entire pathophysiological process yet to be elucidated. We present a case of a 51-year-old male with a history of previously treated multiple myeloma in remission, initially presenting with chest pain with an unremarkable work-up. His hospital course was complicated by hyperammonemia causing encephalopathy requiring mechanical ventilation. After an extensive work-up to find an underlying cause, he was diagnosed with relapsing multiple myeloma. Due to a prolonged and complicated hospital course, the family pursued comfort measures and the patient passed away peacefully. Multiple myeloma induced hyperammonemic encephalopathy is a rare phenomenon carrying a high morbidity and mortality rate. Being still poorly understood, this manifestation of an already lethal diagnosis should be considered as a differential diagnosis of hyperammonemia. While early and aggressive treatment has shown some benefit and improved patient outcomes, further studies and understanding is needed to help diminish the mortality associated with hyperammonemic encephalopathy due to multiple myeloma.

摘要

多发性骨髓瘤是一种浆细胞恶性肿瘤,浆细胞常见于骨髓中。多发性骨髓瘤以引发多种症状并影响多个器官系统而闻名,它是一种罕见的恶性肿瘤,其整个病理生理过程尚待阐明。我们报告一例51岁男性病例,该患者既往有多发性骨髓瘤病史,经治疗后处于缓解期,最初表现为胸痛,各项检查均无异常。其住院过程因高氨血症并发脑病而变得复杂,需要机械通气。在进行广泛检查以寻找潜在病因后,他被诊断为复发性多发性骨髓瘤。由于住院时间长且病情复杂,家属采取了姑息治疗措施,患者最终平静离世。多发性骨髓瘤诱发的高氨血症性脑病是一种罕见现象,发病率和死亡率都很高。由于对此仍了解不足,对于这种已属致命诊断的表现形式,应将其视为高氨血症的鉴别诊断之一。虽然早期积极治疗已显示出一定益处并改善了患者预后,但仍需要进一步研究和了解,以帮助降低多发性骨髓瘤所致高氨血症性脑病的死亡率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/daeb/7891795/7cfd867e9897/cureus-0013-00000012781-i01.jpg

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