Fira-Mladinescu Ovidiu, Suppini Noemi, Olteanu Gheorghe-Emilian, Fira-Mladinescu Corneluta, Traila Daniel
Center for Research and Innovation in Personalised Medicine of Respiratory Diseases, XIIIth Department-Pulmonology Discipline, "Victor Babes" University of Medicine and Pharmacy Timisoara, Eftimie Murgu Sq. no. 2, 300041 Timișoara, Romania.
Center of Expertise for Rare Lung Diseases, Clinical Hospital of Infectious Diseases and Pneumophthisiology "Dr. Victor Babes" Timisoara, Gh. Adam Street no. 13, 300310 Timisoara, Romania.
Diagnostics (Basel). 2022 Jun 4;12(6):1394. doi: 10.3390/diagnostics12061394.
Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse cystic lung disease that occurs almost exclusively in young adult smokers. High-resolution computed tomography of the chest allows a confident diagnosis of PLCH in typical presentation, when nodules, cavitating nodules, and cysts coexist and show a predominance for the upper and middle lung. Atypical presentations require histology for diagnosis. Histologic diagnosis rests on the demonstration of increased numbers of Langerhans cells and/or specific histological changes. PLCH is one of the few diseases in which bronchoalveolar lavage (BAL) has a high diagnostic value and can in some circumstances replace lung biopsy. We present a case of PLCH in an elderly non-smoker. Chest imaging revealed the presence of advanced interstitial lung disease with a fibrocystic pattern. BAL cellular analyses disclosed a macrophage pattern with CD1a phenotype that strongly supports the PLCH diagnosis, even in the setting of atypical clinical presentation and a lack of smoking exposure. PLCH is extremely rare in non-smokers and could represent a distinct phenotype.
肺朗格汉斯细胞组织细胞增多症（PLCH）是一种罕见的弥漫性囊性肺疾病，几乎仅发生于年轻成年吸烟者。当结节、空洞性结节和囊肿共存且以上肺和中肺为主时，胸部高分辨率计算机断层扫描能够在典型表现中确诊PLCH。非典型表现则需要组织学检查来确诊。组织学诊断基于朗格汉斯细胞数量增加和/或特定组织学改变的证实。PLCH是少数几种支气管肺泡灌洗（BAL）具有较高诊断价值且在某些情况下可替代肺活检的疾病之一。我们报告一例老年非吸烟者的PLCH病例。胸部影像学显示存在具有纤维囊性模式的晚期间质性肺疾病。BAL细胞分析显示具有CD1a表型的巨噬细胞模式，即使在非典型临床表现和无吸烟史的情况下，也强烈支持PLCH的诊断。PLCH在非吸烟者中极为罕见，可能代表一种独特的表型。
