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肺朗格汉斯细胞组织细胞增多症的诊断挑战:一例成人结节性表现的病例报告

Diagnostic challenges in pulmonary Langerhans cell histiocytosis: A case report of nodular presentation in an adult.

作者信息

Aziz Nouman, Nabi Waseem, Makhkamov Sukhrob, Kumari Sadhna, Chepkunov Dimitri, Chung Daye, Sahni Sonu

机构信息

Wyckoff Heights Medical Center, Brooklyn, NY, USA.

Jefferson Health Northeast, Philadelphia, PA, USA.

出版信息

Respir Med Case Rep. 2025 Jun 27;57:102245. doi: 10.1016/j.rmcr.2025.102245. eCollection 2025.

Abstract

Adult pulmonary Langerhans cell histiocytosis (PLCH) represents a poorly understood subset of LCH, primarily involving young to middle-aged smokers and accounting for approximately 3-5 % of adult diffuse lung diseases. This case report presents uncommon presentation of adult PLCH with nodular pulmonary lesions on a 40-year-old female with a history of asthma, systemic lupus erythematosus (SLE), and smoking. CT chest confirmed multiple bilateral pulmonary nodules, with the largest measuring up to 12 mm. Biopsy of the lung nodules demonstrated Langerhans cell infiltration, with immunohistochemical staining positive for CD1a and S100, confirming the diagnosis of PLCH. The report underscores the importance of considering PLCH in the differential diagnosis of pulmonary nodules, particularly in patients with a significant smoking history, and contributes to the growing body of knowledge regarding the diverse presentations of LCH in adults.

摘要

成人肺朗格汉斯细胞组织细胞增多症(PLCH)是朗格汉斯细胞组织细胞增多症(LCH)中一个了解较少的亚型,主要累及年轻至中年吸烟者,约占成人弥漫性肺疾病的3%至5%。本病例报告呈现了一名40岁女性成人PLCH的罕见表现,该患者有哮喘、系统性红斑狼疮(SLE)病史且吸烟,胸部CT证实双侧肺部有多个结节,最大直径达12毫米。肺结节活检显示朗格汉斯细胞浸润,免疫组化染色CD1a和S100呈阳性,确诊为PLCH。该报告强调了在肺结节鉴别诊断中考虑PLCH的重要性,尤其是在有大量吸烟史的患者中,并为关于成人LCH多种表现的知识积累做出了贡献。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d25e/12272765/a7a5f757430a/gr1.jpg

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