Intriguing bronchoalveolar lavage proteome in a case of pulmonary langerhans cell histiocytosis

Abstract

Background: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).Case Report: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.Conclusions: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.

The Role of One- and Two-Dimensional Electrophoretic Techniques in Proteomics of the Lung

“…Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease associated with the exposure to tobacco smoke. To provide new insights into its pathogenesis and to compare this disorder with COPD, Ghafouri et al [39] analyzed BALf from a biopsy-proven case of PLCH, a COPD patient, and a healthy control. Proteins were separated by 2-DE, and their patterns were analyzed with a computerized 2-DE imaging system.…”

“…Among others, the authors observed that retinol-binding protein 4 (RBP4) and fetuin-A were significantly lowered in patients with active TB compared to controls, thus suggesting that they could be considered potential biomarkers for monitoring the course of the disease during clinical treatment. [32,[35][36] Lung fibrosis associated to SSc BALf [32,37] RA-ILD BALf [38] PLCH BALf [32,39] CF Serum [41] COPD BALf [29,30,44,46,47] IPAH Serum [48][49][50] TB Serum [61] Electrophoresis -Life Sciences Practical Applications…”