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肠病相关T细胞淋巴瘤作为无症状乳糜泻的一种并发症。

Enteropathy-associated T cell lymphoma as a complication of silent celiac disease.

作者信息

Brito Margarida Dantas, Martins Ângelo, Henrique Rui, Mariz José

机构信息

Department of Hematology.

Department of Pathology, Portuguese Institute of Oncology of Porto , Portugal.

出版信息

Hematol Rep. 2014 Dec 9;6(4):5612. doi: 10.4081/hr.2014.5612. eCollection 2014 Nov 19.

Abstract

Celiac disease is an autoimmune disorder in which a genetic predisposition and the ingestion of wheat gluten triggers a deleterious immune response. This response is complex and may lead to manifestations other than enteropathyha: hepatitis, dermatitis and neuropathy. There is higher risk for neoplasia. We observed an atypical case, corresponding to a 69-year old female presenting with complicated celiac disease. The patient was referred following the histological examination of an enterectomy specimen, which unexpectedly revealed an enteropathy-associated T cell lymphoma in a background of celiac disease. Patient's previous medical history comprised several abdominal surgical procedures, without other prior symptoms suggestive of celiac disease. Indeed, the patient was obese and no signs of malabsortion were apparent. This case draws our attention to clinically silent celiac disease, which represents a diagnostic challenge. Thus, this should be kept in mind whenever a patient presents with abdominal relapsing complications, otherwise unexplained.

摘要

乳糜泻是一种自身免疫性疾病,遗传易感性和摄入小麦麸质会引发有害的免疫反应。这种反应很复杂,可能导致除肠病之外的其他表现,如肝炎、皮炎和神经病变。患肿瘤的风险更高。我们观察到一例非典型病例,是一名69岁患有复杂性乳糜泻的女性。该患者是在对肠切除标本进行组织学检查后前来就诊的,检查意外发现其在乳糜泻背景下存在肠病相关T细胞淋巴瘤。患者既往病史包括多次腹部外科手术,之前没有其他提示乳糜泻的症状。实际上,该患者肥胖,没有明显的吸收不良迹象。这个病例让我们注意到临床无症状的乳糜泻,它是一个诊断挑战。因此,每当患者出现腹部复发性并发症且原因不明时,都应考虑到这一点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c33b/4274481/7bd7af29511d/hr_2014-04-5612-g001.jpg

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