肺部Langerhans’細胞組織細胞增生症是一瀰漫性,和抽煙病史有關的間質性肺疾,病理上的特徵是細小支氣管有向心性發炎,形成囊泡,以及廣泛性的內因性血管病變。雖然有時會自動緩解,對一些臨床上會逐漸惡化的病例,肺臟移植可以做為治療上的選擇。在此,我們報告一28歲男性病例,因為末期的肺部Langerhans’細胞組織細胞增生症而實施右側單肺移植手術。他手術後存活,平安恢復,經6個月追蹤後,肺部功能有顯著改善。
Pulmonary Langerhans’ cell histiocytosis (LCH) is a diffuse, smoking-related, interstitial lung disease characterized pathologically by bronchiolocentric inflammation, cyst formation, and widespread intrinsic vascular abnormalities. Despite spontaneous remissions sometimes occurring, the clinical status progressively worsens, and lung transplantation offers a therapeutic option in a few cases. The present case is a 28-year-old man who underwent right single lung transplantation (SLT) for end-stage pulmonary Langerhans’ cell histiocytosis. He survived the SLT. Postoperative recovery was uneventful, and his respiratory function improved significantly after 6 months of follow-up.