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[一位老年女性的爱泼斯坦-巴尔病毒相关噬血细胞性淋巴组织细胞增生症病例]

[A case of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in an elderly woman].

作者信息

Matsumoto Shoya, Nishihara Keiji, Hasegawa Masaki, Satake Shosuke, Endo Hidetoshi, Arai Hidenori

机构信息

Department of Geriatric Medicine, The University of Tokyo Hospital.

Department of Geriatric Medicine, National Center for Geriatrics and Gerontology.

出版信息

Nihon Ronen Igakkai Zasshi. 2019;56(4):525-531. doi: 10.3143/geriatrics.56.525.

DOI:10.3143/geriatrics.56.525
PMID:31761859
Abstract

An 84-year-old woman who had diabetes mellitus and atrial fibrillation presented to our hospital with appetite loss and difficulty in standing up. Her cervical, axillary, and mediastinal lymph nodes were swollen. At first, malignant lymphoma was suspected, but positron emission tomography-computed tomography and cervical lymph node biopsy did not support this diagnosis. She was admitted to our hospital 41 days after the first consultation because of appetite loss and fever. We suspected a disorder related to Epstein-Barr virus infection because Epstein-Barr virus-encoded small RNAs were found in the lymph node. She was diagnosed with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) based on the diagnostic criteria of the Japanese Society for Pediatric Infectious Diseases. Her symptoms, including appetite loss and fever, were alleviated after the administration of prednisolone (20 mg per day). She was discharged 90 days after admission. At two weeks after discharge, she was readmitted for respiratory failure, and died five days after readmission.To the best of our knowledge there are no previous reports of EBV-HLH in patients over 80 years of age, and the optimal treatment strategy is unknown. This case suggested that prednisolone may alleviate the symptoms of EBV-HLH. When a patient presents with swollen lymph nodes, clinicians should keep the possibility of EBV-HLH in mind.

摘要

一名患有糖尿病和心房颤动的84岁女性因食欲减退和站立困难前来我院就诊。她的颈部、腋窝和纵隔淋巴结肿大。起初怀疑为恶性淋巴瘤,但正电子发射断层扫描-计算机断层扫描及颈部淋巴结活检均不支持该诊断。首次就诊41天后,她因食欲减退和发热入住我院。由于在淋巴结中发现了爱泼斯坦-巴尔病毒编码的小RNA,我们怀疑与爱泼斯坦-巴尔病毒感染相关的疾病。根据日本儿科传染病学会的诊断标准,她被诊断为爱泼斯坦-巴尔病毒相关噬血细胞性淋巴组织细胞增生症(EBV-HLH)。给予泼尼松龙(每日20mg)后,她的食欲减退和发热等症状得到缓解。入院90天后出院。出院两周后,她因呼吸衰竭再次入院,并在再次入院五天后死亡。据我们所知,此前尚无80岁以上患者发生EBV-HLH的报道,最佳治疗策略也未知。该病例提示泼尼松龙可能缓解EBV-HLH的症状。当患者出现淋巴结肿大时,临床医生应考虑到EBV-HLH的可能性。

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