Ghafouri Bijar, Persson H Lennart, Tagesson Christer
Rehabilitation Medicine, Department of Medical and Health Sciences (IMH), Faculty of Health Sciences, Linköping University, Pain- and Rehabilitation Centre, County Council of Östergötland, Linköping, Sweden ; Occupational and Environmental Medicine, Department of Clinical and Experimental Medicine (IKE), Faculty of Health Sciences, Linköping University, Centre of Occupational and Environmental Medicine, County Council of Östergotland, Linkoping, Sweden.
Am J Case Rep. 2013 Apr 29;14:129-33. doi: 10.12659/AJCR.889037. Print 2013.
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease associated with tobacco smoke exposure. New insights into its pathogenesis and how it differs from that of chronic obstructive pulmonary disease (COPD) may be provided by proteomic studies on bronchoalveolar lavage fluid (BALF).
We present the BALF proteome in a biopsy-proven case of PLCH and compare it with typical proteomes of COPD and of the healthy lung. The BALF proteins were separated by two-dimensional gel electrophoresis (2-DE) and the protein patterns were analyzed with a computerized 2-DE imaging system. As compared to the healthy subject and the COPD case, the PLCH case showed a strikingly different 2-DE pattern. There was much more IgG (heavy chain) and orosomucoid, and less α1-antitrypsin, surfactant protein-A, haptoglobin, cystatin-S, Clara cell protein 10, transthyretin and gelsolin. Moreover, no apolipoprotein-A1, pro-apolipoprotein-A1, amyloid P, calgranulin A, or calgranulin B was detected at all.
This case of PLCH presents with an extreme BALF proteome lacking significant amounts of protective and anti-inflammatory proteins. Thus, the intriguing BALF proteome opens up new lines of research into the pathophysiology of PLCH and how its pathogenesis differs from that in COPD.
肺朗格汉斯细胞组织细胞增多症（PLCH）是一种与接触烟草烟雾相关的罕见间质性肺疾病。支气管肺泡灌洗（BALF）蛋白质组学研究可能为其发病机制以及与慢性阻塞性肺疾病（COPD）的差异提供新见解。
我们展示了一例经活检证实的PLCH患者的BALF蛋白质组，并将其与COPD和健康肺脏的典型蛋白质组进行比较。通过二维凝胶电泳（2-DE）分离BALF蛋白质，并用计算机二维成像系统分析蛋白质图谱。与健康受试者和COPD病例相比，PLCH病例显示出截然不同的二维图谱。IgG（重链）和血清类黏蛋白更多，而α1-抗胰蛋白酶、表面活性蛋白A、触珠蛋白、胱抑素-S、克拉拉细胞蛋白10、转甲状腺素蛋白和凝溶胶蛋白更少。此外，完全未检测到载脂蛋白A1、前载脂蛋白A1、淀粉样蛋白P、钙粒蛋白A或钙粒蛋白B。
该PLCH病例呈现出一种极端的BALF蛋白质组，缺乏大量的保护性和抗炎性蛋白。因此，这个有趣的BALF蛋白质组为PLCH的病理生理学及其发病机制与COPD的差异开辟了新的研究方向。
