Department of Hematology, Toyama University Hospital, Japan.
Center for Medical Residency Training, Toyama University Hospital, Japan.
Intern Med. 2022 Jul 1;61(13):2051-2055. doi: 10.2169/internalmedicine.8261-21. Epub 2021 Nov 27.
Protein-losing enteropathy (PLE) is a rare syndrome characterized by hypoproteinemia due to gastrointestinal (GI) protein loss. Primary intestinal follicular lymphoma (PIFL), a specific variant of follicular lymphoma with essential only GI involvement, has not been reported as an etiology of PLE. We herein report a case of PLE complicated with PIFL that was successfully treated with rituximab, resulting in rapid improvement of PLE and a complete response of PIFL. Macroscopic findings of ulcerative lesions with diffuse involvement, which were precisely described by capsule and double-balloon enteroscopy at the diagnosis, also improved following the treatment. This case provides a clue suggesting factors that promote PLE in PIFL.
肠病相关性蛋白丢失症（PLE）是一种罕见的综合征，其特征为因胃肠道（GI）蛋白丢失导致低蛋白血症。原发性肠道滤泡淋巴瘤（PIFL）是滤泡淋巴瘤的一种特殊亚型，仅累及胃肠道，尚未有报道其为 PLE 的病因。本文报告了一例 PLE 合并 PIFL 的病例，该病例使用利妥昔单抗治疗后成功缓解，PLE 迅速改善，PIFL 完全缓解。诊断时胶囊内镜和双气囊小肠镜精确描述的溃疡性病变弥漫性受累的大体发现，在治疗后也得到了改善。该病例提示了 PIFL 中促进 PLE 的因素。
