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从诊断性活检到肺大疱切除术:一例肺朗格汉斯细胞组织细胞增多症的进行性并发症病例

From diagnostic biopsy to bullectomy: A case of progressive complications in pulmonary langerhans cell histiocytosis.

作者信息

Hsin Yuan-Chia, Huang Yung-Chia, Wu Yen-Lin, Lee Shih-Wei, Lo Po-Chun

机构信息

Department of Pulmonary Medicine, Taoyuan General Hospital, Ministry of Health and Welfare, Taoyuan, Taiwan.

Department of Chest Surgery, Taoyuan General Hospital, Ministry of Health and Welfare, Taoyuan, Taiwan.

出版信息

Respir Med Case Rep. 2025 Jul 19;57:102263. doi: 10.1016/j.rmcr.2025.102263. eCollection 2025.

Abstract

A 38-year-old male smoker presented with a persistent dry cough lasting several weeks. Chest radiography showed bilateral reticulonodular opacities predominantly in the upper lung fields. Subsequent chest computed tomography revealed diffuse, thick-walled, irregular cystic lesions mainly involving the mid and upper lung zones with a centrilobular distribution. Histopathological examination obtained through video-assisted thoracoscopic surgery demonstrated bronchiolocentric nodules and cysts containing Langerhans cells with convoluted nuclei, confirmed by positive immunohistochemical staining for CD1a, S-100, and Langerin. These findings confirmed the diagnosis of pulmonary Langerhans Cell Histiocytosis (PLCH). The patient's symptoms improved initially following smoking cessation but were complicated by recurrent pneumothorax and the formation of giant bullae, ultimately requiring surgical intervention. This case emphasizes the association between PLCH and smoking, highlights potential complications following surgical biopsy, and underscores smoking cessation as a critical therapeutic measure, with targeted therapies reserved for refractory or mutation-specific cases.

摘要

一名38岁男性吸烟者出现持续数周的干咳。胸部X线检查显示双侧网状结节状阴影,主要位于上肺野。随后的胸部计算机断层扫描显示弥漫性、厚壁、不规则囊性病变,主要累及中、上肺区,呈小叶中心分布。通过电视辅助胸腔镜手术获得的组织病理学检查显示支气管中心性结节和含有核呈卷曲状的朗格汉斯细胞的囊肿,CD1a、S-100和朗格素免疫组化染色阳性证实了这一点。这些发现确诊为肺朗格汉斯细胞组织细胞增多症(PLCH)。患者戒烟后症状最初有所改善,但并发反复气胸和巨大肺大疱形成,最终需要手术干预。该病例强调了PLCH与吸烟之间的关联,突出了手术活检后的潜在并发症,并强调戒烟是一项关键的治疗措施,靶向治疗仅适用于难治性或特定突变病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4544/12309013/4357b318a568/gr1.jpg

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