结外NK/ t细胞淋巴瘤表现为致死性胃肠道出血和噬血细胞性淋巴组织细胞增多症。

IF 0.6 Q3 MEDICINE, GENERAL & INTERNAL
Neha Maini, Kathryn Biddle, Anna Green, Michelle Fernando, Giovanni Sanna
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引用次数: 0

摘要

嗜血球性淋巴组织细胞增多症(HLH)是一种严重的高炎症综合征,在持续发烧的患者中应予以考虑。在这里,我们提出了一个临床诊断为behaperet病的病人,他表现为急性直肠出血,继发于急性腔内回肠出血。在入院期间,她出现了伴有发热、高铁血症、细胞减少和凝血功能障碍的HLH。尽管静脉注射了皮质类固醇和阿那白,但她的病情迅速恶化,16天后死亡。她死后,根据骨髓活检和肠组织病理学诊断为结外NK/ t细胞淋巴瘤。本病例强调了发热、细胞计数下降和高铁血症患者早期考虑HLH和确定潜在病因的重要性。通过本病例报告,我们讨论了诊断的挑战和多学科方法的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Extranodal NK/T-cell lymphoma presenting as fatal gastrointestinal bleeding and haemophagocytic lymphohistiocytosis.

Haemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome that should be considered in patients with persistent fevers. Here, we present a patient with a clinical diagnosis of Behçet's disease who presented acutely with rectal bleeding secondary to acute intraluminal ileal haemorrhage. During her admission, she developed HLH with fevers, hyperferritinaemia, cytopaenia and coagulopathy. Despite treatment with intravenous corticosteroids and anakinra, her condition deteriorated rapidly, and she died 16 days later. After her death, the diagnosis of extranodal NK/T-cell lymphoma was made based on bone marrow biopsy and bowel histopathology. This case highlights the importance of early consideration of HLH in patients with fevers, falling cell counts and hyperferritinaemia and identification of the underlying aetiology. Through this case report, we discuss the diagnostic challenges and importance of a multidisciplinary approach.

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来源期刊
BMJ Case Reports
BMJ Case Reports Medicine-Medicine (all)
CiteScore
1.40
自引率
0.00%
发文量
1588
期刊介绍: BMJ Case Reports is an important educational resource offering a high volume of cases in all disciplines so that healthcare professionals, researchers and others can easily find clinically important information on common and rare conditions. All articles are peer reviewed and copy edited before publication. BMJ Case Reports is not an edition or supplement of the BMJ.
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