Indolent T cell lymphoproliferative disorder with villous atrophy in small intestine diagnosed by single-balloon enteroscopy

Takashi Nagaishi,Kohei Suzuki,Kouhei Yamamoto,Kengo Takeuchi,Masayoshi Fukuda,Mamoru Watanabe,Ryosuke Fukuyo,Naoya Tsugawa,Daiki Yamada,Kazuo Ohtsuka,Eiko Saito,Taro Watabe,Ayako Arai

doi:10.1007/s12328-019-00971-1

Abstract

Chronic diarrhea is one of the major symptoms in gastroenterology. However, this may be caused by pathologic conditions for which the diagnosis is critical. Villous atrophy, as an endoscopic lesion, accompanied by chronic diarrhea can occasionally be observed in the patients with inflammatory diseases of the gastrointestinal (GI) tract. Herein, we present a case with persistent diarrhea accompanied by intestinal wall thickening without any other significant endoscopic features other than villous atrophy in the jejunum and the ileum, where we diagnosed as an indolent T cell lymphoproliferative disorder (T-LPD) of the GI tract, defined in the 2016–2017 revised World Health Organization classification, via single-balloon enteroscopy (SBE). Interestingly, we found the same lymphocyte infiltration from the distal third portion of the duodenum, where gastroscopy could not reach, via SBE, even though no endoscopic findings were observed such as villous atrophy. Since infiltrating cells in the intestinal tissues were CCR4+, mogamulizumab was administered with resulting durable symptomatic remission for more than 2 years. Patients with persistent diarrhea may have serious small intestinal disorder including not only chronic inflammatory diseases but also lymphoid neoplasmic conditions including T-LPD of GI tract.

Highlights

Diarrhea is one of the major symptoms in the field of gastroenterology, and many cases are caused by inflammation due to infection with pathogens such as viruses and bacteria [1]
Those with long standing diarrhea are often diagnosed as irritable bowel syndrome (IBS) when other specific inflammation and/or infection are ruled out
Villous atrophy can occasionally be observed under endoscopic studies in patients with persistent diarrhea, especially in those suffering from inflammatory diseases of the gastrointestinal (GI) tract such as celiac disease, Crohn’s disease, eosinophilic gastroenteritis and familial Mediterranean fever [2,3,4,5]

Summary

Introduction

Diarrhea is one of the major symptoms in the field of gastroenterology, and many cases are caused by inflammation due to infection with pathogens such as viruses and bacteria [1]. We present a case with persistent diarrhea without any other significant endoscopic findings other than villous atrophy in the small intestine where we diagnosed one form of lymphoproliferative disease using single-balloon enteroscopy (SBE). He had moved to a Southeast Asian country 12 years earlier, and he started having persistent diarrhea 2 years later He came back to Japan to visit an outside hospital and was diagnosed as IBS, because stool culture and colonoscopy were normal, and the parasite infection studies were negative. Four years later, his diarrhea worsened with occasional fever. There was no response in either clinical or histological presentations even after 6

Discussion

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