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成人非吸烟者骨朗格汉斯细胞组织细胞增多症伴肺部受累:一例报告及文献简要综述

Bone Langerhans cell histiocytosis with pulmonary involvement in an adult non-smoker: A case report and brief review of the literature.

作者信息

Shen Jie, Feng Shicheng

机构信息

Department of Imaging, Nanjing Chest Hospital, Medical School of Southeast University, Nanjing, Jiangsu 210009, P.R. China.

Department of Oncology, Zhongda Hospital Affiliated to Southeast University, Nanjing, Jiangsu 210009, P.R. China.

出版信息

Mol Clin Oncol. 2017 Jan;6(1):67-70. doi: 10.3892/mco.2016.1077. Epub 2016 Nov 10.

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease of unknown cause, which encompasses a set of disorders of multiple organs with various clinical presentations that share the common characteristic of sizeable Langerhans cell infiltration. The clinical spectrum of LCH ranges from solitary bone lesions to involvement of other organs. lung involvement in LCH may be part of a multisystem disease, which almost exclusively occurs in adult smokers, while it is overlooked or misdiagnosed in the majority of non-smokers. High-resolution computed tomography (HRCT) of the chest is crucial for diagnosis; however, the treatment and prognosis of this disease have not been clearly determined. We herein present the case of a non-smoking adult patient who presented with lower limb pain and was diagnosed via biopsy with lch with multisystem involvement, including the bone and lungs. Lytic lesions in the corpus of the sacroiliac joint, sacrum, acetabulum and femoral head by a soft tissue mass were observed on diagnostic CT. In addition, chest HRCT revealed multiple cysts in the bilateral lungs, predominantly in the upper lobes. The final diagnosis of LCH was confirmed by histopathological examination and immunohistochemical staining for CD1a and S-100. Corticosteroid treatment alleviated lower limb pain and improved the patient's quality of life; thus, corticosteroids may be considered as a potential treatment option for patients with LCH.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种病因不明的罕见疾病,它包括一组多器官疾病,具有多种临床表现,共同特征是有大量朗格汉斯细胞浸润。LCH的临床谱从孤立性骨病变到其他器官受累不等。LCH的肺部受累可能是多系统疾病的一部分,几乎仅发生于成年吸烟者,而在大多数非吸烟者中易被忽视或误诊。胸部高分辨率计算机断层扫描(HRCT)对诊断至关重要;然而,该疾病的治疗和预后尚未明确确定。我们在此报告一例非吸烟成年患者,该患者因下肢疼痛就诊,经活检诊断为LCH伴多系统受累,包括骨骼和肺部。诊断性CT显示骶髂关节、骶骨、髋臼和股骨头体部有溶骨性病变伴软组织肿块。此外,胸部HRCT显示双侧肺部有多个囊肿,主要位于上叶。通过组织病理学检查以及CD1a和S-100免疫组化染色确诊为LCH。皮质类固醇治疗缓解了下肢疼痛并改善了患者的生活质量;因此,皮质类固醇可被视为LCH患者的一种潜在治疗选择。

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