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普通可变免疫缺陷综合征中的结节性淋巴样增生在内镜检查时酷似家族性腺瘤性息肉病。

Nodular lymphoid hyperplasia in common variable immunodeficiency syndrome mimicking familial adenomatous polyposis on endoscopy.

作者信息

Molaei Mahsa, Kaboli Alireza, Fathi Alireza Modares, Mashayekhi Reza, Pejhan Shervin, Zali Mohammad Reza

机构信息

Department of Pathology and Gastroenterology, Research Center for Gastroenterology and Liver Disease, Shaheed Beheshti University MC, Tehran, Iran.

出版信息

Indian J Pathol Microbiol. 2009 Oct-Dec;52(4):530-3. doi: 10.4103/0377-4929.56152.

Abstract

Common variable immunodeficiency syndrome (CVID) includes a heterogeneous disorder characterized by reduced levels of IgG, IgA or IgM, and recurrent bacterial infections with normal T-cell immunity in 60% of patients. It affects the gastrointestinal tract as the largest immune organ with a wide spectrum of symptoms and signs. We present a case of nodular lymphoid hyperplasia (NLH) of the small intestine in a 31-year-old man admitted for evaluation of chronic diarrhea. Upper and lower gastrointestinal endoscopy revealed multiple polyps in the stomach, duodenum, ileum, and large intestine mimicking familial adenomatous polyposis (FAP). Although he had no history of recurrent infection, immunological profiles were in favor of CVID. We emphasize the importance of considering CVID in any patient with gastrointestinal manifestations even in the absence of recurrent bacterial infections. Diagnostic delay results in more morbidity and complications in untreated patients.

摘要

普通可变免疫缺陷综合征(CVID)是一种异质性疾病,其特征为IgG、IgA或IgM水平降低,60%的患者T细胞免疫正常但反复发生细菌感染。它作为最大的免疫器官影响胃肠道,有广泛的症状和体征。我们报告一例31岁男性小肠结节性淋巴组织增生(NLH)病例,该患者因慢性腹泻入院评估。上消化道和下消化道内镜检查发现胃、十二指肠、回肠和大肠有多个息肉,酷似家族性腺瘤性息肉病(FAP)。尽管他没有反复感染史,但免疫检查结果支持CVID诊断。我们强调,即使没有反复细菌感染,对于任何有胃肠道表现的患者都要考虑CVID的可能性。诊断延误导致未治疗患者出现更多的发病率和并发症。

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