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[两例单形上皮样肠道T细胞淋巴瘤致死病例在肠道穿孔急诊手术后迅速恶化]

[Two Lethal Cases of Monomorphic Epitheliotropic Intestinal T-cell Lymphoma Deteriorated Rapidly After Emergency Surgery for Intestinal Perforation].

作者信息

Ko Sanguk, Park Sang Hyoung

机构信息

Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

出版信息

Korean J Gastroenterol. 2021 Jul 25;78(1):53-58. doi: 10.4166/kjg.2021.046.

Abstract

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive form of primary gastrointestinal T-cell lymphoma. Symptoms can vary but often include fever, abdominal pain, weight loss, diarrhea, obstruction, and perforation. Disease-specific symptoms rarely present before patients reach an advanced stage, which contributes to delayed diagnosis and poor survival outcomes. Approximately half of the patients with MEITL undergo emergency surgery for acute intestinal obstruction or perforation, leading to peritonitis, septic shock, and multiple organ failure. These factors contribute to treatment delays, which are associated with a worse prognosis, particularly in the case of chemotherapy. This paper reports two fatal cases of patients with MEITL who deteriorated rapidly after emergency surgery for intestinal perforation. Patient 1 complained of persistent diarrhea, but a delayed diagnosis led to bowel perforation, and the subsequent chemotherapy treatment was canceled. Patient 2 was diagnosed relatively early, but treatment was delayed due to intestinal perforation. Despite its rarity, MEITL should be considered through a "high index of suspicion" approach when a patient complains of unexplained abdominal pain and diarrhea. This is expected to improve early diagnosis and ultimately patient prognosis.

摘要

单形性上皮趋化性肠道T细胞淋巴瘤(MEITL)是原发性胃肠道T细胞淋巴瘤的一种罕见且侵袭性的形式。症状可能各不相同,但通常包括发热、腹痛、体重减轻、腹泻、肠梗阻和穿孔。特定疾病的症状在患者进入晚期之前很少出现,这导致诊断延迟和生存结果不佳。大约一半的MEITL患者因急性肠梗阻或穿孔接受急诊手术,导致腹膜炎、感染性休克和多器官功能衰竭。这些因素导致治疗延迟,而治疗延迟与预后较差相关,尤其是在化疗方面。本文报告了两例MEITL患者的致命病例,他们在因肠穿孔进行急诊手术后迅速恶化。患者1主诉持续腹泻,但诊断延迟导致肠穿孔,随后的化疗治疗被取消。患者2诊断相对较早,但由于肠穿孔治疗延迟。尽管MEITL罕见,但当患者主诉不明原因的腹痛和腹泻时,应通过“高度怀疑”的方法来考虑。这有望改善早期诊断并最终改善患者预后。

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