症状与炎症性肠病相似的肠朗格汉斯细胞组织细胞增生症:病例报告

Yuqing Liu, Zhenwei Chen, Lu Wang, Baizhou Li
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摘要

朗格汉斯细胞组织细胞增生症是一种罕见疾病,其特征是朗格汉斯细胞在单个器官或多个器官内异常增生。本病例报告旨在提高人们对胃肠道朗格汉斯细胞组织细胞增生症表现的认识,以促进这种罕见疾病的诊断和治疗。腹部超声波检查发现脾脏略有肿大。最初的结肠镜检查显示她患有慢性肠炎,并伴有早期炎症性肠病。抗炎治疗无好转后,在浙江大学附属第四医院进行了肠活检。最终的肠活检和组织病理学检查证实了朗格汉斯细胞组织细胞增生症的存在。确诊后,额外的肺部和头部影像学检查未发现异常。在接受化疗(长春新碱和泼尼松)和分子靶向药物(达拉非尼)治疗后,她的病情逐渐好转。因此,如果婴儿出现炎症性胃肠道症状,且经治疗后症状仍未缓解,则必须进行活组织检查以获得鉴别诊断。
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Intestinal Langerhans cell histiocytosis presenting with symptoms similar to inflammatory bowel disease: a case report
Langerhans cell histiocytosis is a rare disease characterized by the abnormal proliferation of Langerhans cells within a single organ or multiple organs. This case report aims to improve the knowledge of the presentation of gastrointestinal Langerhans cell histiocytosis to facilitate the diagnosis and management of this rare disorder.A 19-month-old female presented with repeatedly mucinous bloody stools. The abdominal ultrasound revealed a slightly enlarged spleen. The initial colonoscopy revealed chronic enteritis with a very early onset inflammatory bowel disease. After anti-inflammatory treatment without improvement, an intestinal biopsy was performed at The Forth Affiliated Hospital of Zhejiang University. The final intestinal biopsy and histopathology examination confirmed the presence of Langerhans cell histiocytosis. After diagnosis, additional lung and head imaging examinations revealed no abnormalities. Her condition improved gradually after being treated with chemotherapy (vincristine and prednisone) and molecular-targeted drug(dalafinil) treatment.The clinical symptoms of Langerhans cell histiocytosis involving the gastrointestinal tract are not specific and may resemble symptoms observed in inflammatory bowel disease and other primary gastrointestinal tumors. Therefore, in cases of infants presenting with inflammatory gastrointestinal symptoms that do not resolve after treatment, a biopsy is essential to obtain a differential diagnosis.
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