Sholl Lynette M, Hornick Jason L, Pinkus Jack L, Pinkus Geraldine S, Padera Robert F
Department of Pathology, Brigham and Women's Hospital, and Harvard Medical School, Boston, MA 02115, USA.
Am J Surg Pathol. 2007 Jun;31(6):947-52. doi: 10.1097/01.pas.0000249443.82971.bb.
Pulmonary Langerhans cell histiocytosis (LCH) is an idiopathic condition affecting predominantly adult smokers. Histologically, LCH is characterized by a nodular, interstitial proliferation of Langerhans cells around the distal airways with associated eosinophils, lymphocytes, and macrophages. Associated findings, such as fibrosis, emphysematous change, and bronchiolitis can be reminiscent of other interstitial lung diseases. The markers CD1a and S100 have traditionally been used to distinguish LCH from other processes. Little is known about expression of the Langerhans cell-specific lectin, langerin, in pulmonary diseases. We examined the expression patterns of S100, CD1a, and langerin in LCH and other interstitial, inflammatory, and infectious processes in cases retrieved from the files at Brigham and Women's Hospital Department of Pathology. Immunoreactivity was scored according to the number of cells staining per high power field (400x) in areas of highest density, averaged over 4 fields. Cases diagnosed as LCH based on histomorphology and positive CD1a and S100 staining demonstrated strong langerin positivity in lesional tissue. All cases of LCH contained greater than 30 langerin and CD1a positive cells per high power field (HPF), with a mean of >100 cells per HPF, in lesional tissue. Of the other interstitial processes examined, only usual interstitial pneumonia demonstrated increased number of Langerhans cells within epithelium and interstitium (mean 14 cells per HPF) as compared with normal lung (mean 6 cells per HPF). Langerin and CD1a serve as specific diagnostic markers in distinguishing LCH from other interstitial and inflammatory processes.
肺朗格汉斯细胞组织细胞增多症（LCH）是一种主要影响成年吸烟者的特发性疾病。从组织学上看，LCH的特征是在远端气道周围出现朗格汉斯细胞的结节状、间质性增殖，并伴有嗜酸性粒细胞、淋巴细胞和巨噬细胞。相关表现，如纤维化、肺气肿改变和细支气管炎，可能让人联想到其他间质性肺病。传统上，标志物CD1a和S100被用于将LCH与其他病变区分开来。关于朗格汉斯细胞特异性凝集素langerin在肺部疾病中的表达知之甚少。我们在布莱根妇女医院病理科存档的病例中，研究了LCH以及其他间质性、炎症性和感染性病变中S100、CD1a和langerin的表达模式。根据高密度区域每高倍视野（400倍）染色的细胞数量进行免疫反应性评分，在4个视野中取平均值。根据组织形态学以及CD1a和S100染色阳性诊断为LCH的病例，其病变组织中langerin呈强阳性。所有LCH病例的病变组织中，每高倍视野（HPF）含有超过30个langerin和CD1a阳性细胞，平均每HPF>100个细胞。在所检查的其他间质性病变中，与正常肺组织（平均每HPF 6个细胞）相比，只有寻常型间质性肺炎在上皮和间质内的朗格汉斯细胞数量增加（平均每HPF 14个细胞）。Langerin和CD1a作为特异性诊断标志物，可用于将LCH与其他间质性和炎症性病变区分开来。
