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肺朗格汉斯细胞组织细胞增多症伴颈部淋巴结受累,与肺结核及右侧气胸并存:一例报告并文献复习

Pulmonary Langerhans cell histiocytosis with cervical lymph node involvement, and coexistence with pulmonary tuberculosis and right pneumothorax: a case report and review of literature.

作者信息

Gao Limin, Li Huifang, Li Gandi, Liu Weiping, Li Jinnan, Zhang Wenyan

机构信息

Department of Pathology, West China Hospital, Sichuan University Chengdu 610041, China.

Department of Tumor Biotherapy, West China Hospital, Sichuan University Chengdu 610041, China.

出版信息

Int J Clin Exp Pathol. 2015 Feb 1;8(2):2146-52. eCollection 2015.

Abstract

We report an uncommon 22-year-old male Pulmonary Langerhans Cell Histiocytosis (PLCH) case which co-existed with pulmonary tuberculosis (TB). Unlike the common PLCH cases, this PLCH case has cervical lymph node involvement and right pneumothorax. The diagnosis was established by the imaging of lung and the biopsies of the lung and left neck lymph node. Imaging of the chest showed characteristic small nodules and thin-walled cysts and right pneumothorax. The LCH cells in the lung and left neck lymph node were characterized by large convoluted nuclei with cerebriform indentations of the nuclear envelope and longitudinal grooves. The nuclei contained small eosinophilic nucleoli and moderate amount cytoplasm. Immunohistochemically, the histiocytoid cells were positive for Langerin, CD1a and S-100. Acid-fast bacilli were found in sputum and lung biopsy tissue. To the best of our knowledge, this is the first case of PLCH with cervical lymph node involvement, and coexisted with pulmonary tuberculosis, right pneumothorax. A contribution of this case and review three of the five cases of PLCH with extrapulmonary involvement to lymph nodes resolved spontaneously after smoking cessation constitute a novel addition that it is inappropriate to regard pulmonary/nodal LCH as multi-organ or disseminated disease, and the treatment methods are the same whether the PLCH patient with lymph node involvement or not.

摘要

我们报告了一例罕见的22岁男性肺朗格汉斯细胞组织细胞增多症(PLCH)病例,该病例与肺结核(TB)并存。与常见的PLCH病例不同,该PLCH病例有颈部淋巴结受累及右侧气胸。通过肺部影像学检查以及肺和左颈部淋巴结活检确诊。胸部影像学检查显示有特征性的小结节、薄壁囊肿及右侧气胸。肺和左颈部淋巴结中的朗格汉斯细胞组织细胞增多症(LCH)细胞的特征为核大且呈卷曲状,核膜有脑回状切迹及纵沟。细胞核内有小的嗜酸性核仁,胞质中等量。免疫组化显示,组织细胞样细胞朗格蛋白、CD1a和S-100呈阳性。痰及肺活检组织中发现抗酸杆菌。据我们所知;这是首例有颈部淋巴结受累且并存肺结核及右侧气胸 的PLCH病例。该病例以及对五例有肺外淋巴结受累的PLCH病例中的三例进行的回顾表明,戒烟后淋巴结受累自发缓解,这一新发现表明,将肺/淋巴结朗格汉斯细胞组织细胞增多症视为多器官或播散性疾病是不合适的,无论PLCH患者有无淋巴结受累,其治疗方法相同。

相似文献

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Pulmonary langerhans cell histiocytosis.肺朗格汉斯细胞组织细胞增生症。
Orphanet J Rare Dis. 2012 Mar 19;7:16. doi: 10.1186/1750-1172-7-16.
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Adult pulmonary Langerhans' cell histiocytosis.成人肺朗格汉斯细胞组织细胞增多症。
Eur Respir J. 2006 Jun;27(6):1272-85. doi: 10.1183/09031936.06.00024004.
10
Spontaneous pneumothorax.自发性气胸
N Engl J Med. 2000 Mar 23;342(12):868-74. doi: 10.1056/NEJM200003233421207.

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