Department of General Medicine, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, 4-6-1 Shirokanedai, Minato-ku, Tokyo, 108-8639, Japan.
Department of Hematology/Oncology, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, 4-6-1 Shirokanedai, Minato-ku, Tokyo, 108-8639, Japan.
Int J Clin Oncol. 2020 Nov;25(11):1945-1950. doi: 10.1007/s10147-020-01739-1. Epub 2020 Sep 8.
Langerhans cell histiocytosis (LCH) is a rare disease primarily occurring in children, and commonly involves the bone and skin; gastrointestinal tract involvement is notably rare. The incidence and significance of gastrointestinal lesions in adult LCH are unclear; thus, we aimed to investigate adult Japanese cases of LCH and clarify the features of gastrointestinal involvement.
We gathered clinical information on 43 Japanese cases of adult LCH and analyzed patient backgrounds, affected organs, features of the gastrointestinal lesions, and the clinical courses.
Thirteen patients underwent endoscopic examinations: an upper gastrointestinal endoscopy alone in 5, lower gastrointestinal endoscopy alone in 3, and both in 5 patients. A gastric lesion (one case), colonic lesion (one case), and both gastric and rectal lesions (one case) were detected. The three cases of gastrointestinal involvement also exhibited nongastrointestinal multisystem LCH lesions and showed no gastrointestinal symptoms or increased uptake on positron emission tomography. Endoscopy revealed small erosions without specific features; histological examinations were required for diagnosis. These three cases were treated with chemotherapy, comprising vinblastine/prednisolone, methotrexate, and daily 6-mercaptopurine, for 36 weeks; in two cases, the clinical condition remained stable for several years post-treatment. One case showed recurrence 1 year 7 months after treatment, and chemotherapy was re-administered. No case with single-system disease exhibited gastrointestinal involvement.
Although gastrointestinal LCH lesions are rare, they were more common than expected in our cases of multisystem LCH. However, these lesions were relatively small and did not affect the patients' clinical courses.
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见疾病，主要发生在儿童中，常见累及骨骼和皮肤；胃肠道受累则明显罕见。成人 LCH 中胃肠道病变的发病率和意义尚不清楚；因此，我们旨在研究日本成人 LCH 病例并阐明胃肠道受累的特征。
我们收集了 43 例日本成人 LCH 病例的临床资料，并分析了患者背景、受累器官、胃肠道病变特征以及临床病程。
13 例患者接受了内镜检查：5 例单独行上消化道内镜检查，3 例单独行下消化道内镜检查，5 例同时行上、下消化道内镜检查。发现胃病变（1 例）、结肠病变（1 例）和胃直肠均有病变（1 例）。3 例胃肠道受累的病例还表现出非胃肠道多系统 LCH 病变，且无胃肠道症状或正电子发射断层扫描摄取增加。内镜检查显示无特异性特征的小糜烂；需要进行组织学检查以做出诊断。这 3 例患者接受了包含长春新碱/泼尼松、甲氨蝶呤和每日 6-巯基嘌呤的化疗，共 36 周；2 例在治疗后数年病情仍稳定。1 例在治疗后 1 年 7 个月复发，再次接受化疗。单系统疾病无一例出现胃肠道受累。
尽管胃肠道 LCH 病变罕见，但在我们的多系统 LCH 病例中比预期更常见。然而，这些病变相对较小，并未影响患者的临床病程。
