Ahmad Affandi K, Abd Shukor N, Mohamed Rose I, Raja Ali R A, Masir N
Universiti Kebangsaan Malaysia Medical Centre, Department of Pathology, Kuala Lumpur, Malaysia.
Malays J Pathol. 2019 Apr;41(1):65-69.
Coeliac disease enteropathy is associated with an increased risk of lymphomas. Enteropathy-associated T-cell lymphoma is the principal malignancy related to coeliac disease. However, studies have shown that other types of lymphoma such as diffuse large B-cell lymphoma may also be associated with coeliac disease.
We report a 54-year-old Caucasian man who presented with chronic diarrhoea and weight loss. He was diagnosed with coeliac disease based on positive serology results and duodenal, jejunal, and ileal biopsies that showed villous atrophy. Despite adherence to a gluten-free diet, there was no clinical remission and enteropathy-associated T cell lymphoma was suspected. Repeated endoscopic biopsy showed persistent mucosal disease but no evidence of lymphoma. Several weeks later he presented with a perforated jejunum. Histology of the resected jejunum showed diffuse infiltration of submucosa and muscularis propria by malignant lymphoid cells sparing the mucosa. The cells expressed CD20, CD79α, CD10 and BCL6 and ki67 of 80%, consistent with diffuse large B-cell lymphoma.
It is suspected that the undetected lymphoma may have contributed to the persistent malabsorption syndrome rendering the patient unresponsive to treatment. Despite thorough clinical and endoscopic evaluation and multiple biopsies, histologic diagnosis of DLBCL was only confirmed following resection of the perforated jejunum.
乳糜泻肠病与淋巴瘤风险增加相关。肠病相关T细胞淋巴瘤是与乳糜泻相关的主要恶性肿瘤。然而，研究表明，其他类型的淋巴瘤，如弥漫性大B细胞淋巴瘤，也可能与乳糜泻有关。
我们报告一名54岁的白种男性，他出现慢性腹泻和体重减轻。基于血清学结果阳性以及十二指肠、空肠和回肠活检显示绒毛萎缩，他被诊断为乳糜泻。尽管坚持无麸质饮食，但临床并未缓解，怀疑患有肠病相关T细胞淋巴瘤。重复内镜活检显示黏膜疾病持续存在，但无淋巴瘤证据。几周后，他出现空肠穿孔。切除的空肠组织学检查显示黏膜下层和固有肌层被恶性淋巴细胞弥漫浸润，黏膜未受累。这些细胞表达CD20、CD79α、CD10和BCL6，ki67为80%，符合弥漫性大B细胞淋巴瘤。
怀疑未被检测到的淋巴瘤可能导致了持续的吸收不良综合征，使患者对治疗无反应。尽管进行了全面的临床和内镜评估以及多次活检，但直到切除穿孔的空肠后才确诊弥漫性大B细胞淋巴瘤的组织学诊断。
