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伴有颅内转移的肠病相关T细胞淋巴瘤(EATL):一种罕见且预后不良的疾病。

Enteropathy-associated T-cell Lymphoma (EATL) with intracranial metastasis : a rare and dismal condition.

作者信息

Chuah Y Y, Tashi T, Lee Y Y, Fu T Y, Shih C A

机构信息

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Ping Tung Christian Hospital, Ping Tung, Taiwan.

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.

出版信息

Acta Gastroenterol Belg. 2020 Jan-Mar;83(1):77-80.

Abstract

BACKGROUND

Enteropathy-associated T-cell lymphoma (EATL) is a rare type of gastrointestinal non-Hodgkin's Lymphoma. EATL with intracranial metastasis is even rarer. We report a case of EATL with intracranial metastasis.

CASE PRESENTATION

A 36-years old man presented with five weeks history of intractable diarrhea. Colonoscopy was normal, but abdominal computed tomography (CT) scan revealed mural thickening at duodenojejunal junction, and subsequent jejunofiberoscopy showed a circumferential ulceration at the jejunum. Histo-immunopathology confirmed the diagnosis of enteropathyassociated T-cell lymphoma (EATL) type II. His disease course proved to be aggressive and refractory to standard front-line chemotherapy, and eventually progressed through second-line salvage regimen with CNS and intracranial involvement. He died nine months after the initial diagnosis.

CONCLUSION

EATL with brain metastasis is a very rare occurrence with dismal prognosis.

摘要

背景

肠病相关T细胞淋巴瘤(EATL)是一种罕见的胃肠道非霍奇金淋巴瘤。伴有颅内转移的EATL更为罕见。我们报告一例伴有颅内转移的EATL病例。

病例介绍

一名36岁男性,有5周的顽固性腹泻病史。结肠镜检查正常,但腹部计算机断层扫描(CT)显示十二指肠空肠交界处肠壁增厚,随后的空肠纤维镜检查显示空肠有环形溃疡。组织免疫病理学确诊为II型肠病相关T细胞淋巴瘤(EATL)。他的病程进展迅速,对标准一线化疗耐药,最终在二线挽救方案治疗过程中出现中枢神经系统和颅内受累。初诊9个月后死亡。

结论

伴有脑转移的EATL非常罕见,预后不良。

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