Ni Guangsheng, Ou Yaxuan, Ming Runyu, Yang Jin
Department of Thoracic and Cardiovascular Surgery, The First Affiliated Hospital of Hunan University of Traditional Chinese Medicine, Changsha, China.
AME Case Rep. 2025 Jul 15;9:106. doi: 10.21037/acr-24-251. eCollection 2025.
Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm characterized by the abnormal proliferation of Langerhans cells, which can infiltrate various tissues throughout the body, leading to a spectrum of organ damage. This study aims to explore the clinical characteristics, imaging manifestations, and pathological features of the disease to enhance clinical diagnosis and understanding of such conditions.
This study reviews the clinical data of four patients diagnosed with LCH at The First Affiliated Hospital of Hunan University of Traditional Chinese Medicine. All patients met the diagnostic criteria outlined in the LCH guidelines by the American Society of Hematology, with Langerhans cells expressing CD1a, S-100, and Langerin (CD207).
LCH can involve various organs and systems, presenting with diverse clinical manifestations; in particular, rib Langerhans cell histiocytosis (RLCH) primarily manifests as asymptomatic or mildly painful bone swelling. Computerized tomography (CT) imaging of RLCH typically reveals localized masses and focal bone destruction, with or without surrounding soft tissue invasion. Conversely, magnetic resonance imaging provides a clearer assessment of lesion size and the extent of adjacent soft tissue involvement, offering advantages in guiding surgical excision. Diagnosis requires correlation with pathological and immunohistochemical results. For single-system, single-site (SS-s LCH), R0 surgical resection is feasible; however, single-system, multi-site (SS-m LCH) cases necessitate combined chemotherapy or targeted therapies. Overall, the treatment outcomes for this disease remain reasonably favorable.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的组织细胞肿瘤，其特征为朗格汉斯细胞异常增殖，可浸润全身各组织，导致一系列器官损害。本研究旨在探讨该疾病的临床特征、影像学表现及病理特征，以提高临床诊断及对此类病症的认识。
本研究回顾了湖南中医药大学第一附属医院确诊为LCH的4例患者的临床资料。所有患者均符合美国血液学会LCH指南中概述的诊断标准，朗格汉斯细胞表达CD1a、S-100和朗格素（CD207）。
LCH可累及多个器官和系统，临床表现多样；尤其是肋骨朗格汉斯细胞组织细胞增多症（RLCH）主要表现为无症状或轻度疼痛的骨质肿胀。RLCH的计算机断层扫描（CT）成像通常显示局部肿块和局灶性骨质破坏，有无周围软组织侵犯。相反，磁共振成像能更清晰地评估病变大小及相邻软组织受累程度，在指导手术切除方面具有优势。诊断需结合病理及免疫组化结果。对于单系统、单部位（SS-s LCH），R0手术切除可行；然而，单系统、多部位（SS-m LCH）病例需要联合化疗或靶向治疗。总体而言，该疾病的治疗效果仍较为良好。
