Radzikowska Elzbieta
III Department of Lung Diseases and Oncology, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland.
Front Med (Lausanne). 2021 Mar 8;7:582581. doi: 10.3389/fmed.2020.582581. eCollection 2020.
Pulmonary Langerhans cell (LC) histiocytosis (PLCH) has unknown cause and is a rare neoplastic disorder characterized by the infiltration of lungs and various organs by bone marrow-derived Langerhans cells with an accompanying strong inflammatory response. These cells carry somatic mutations of gene and/or , and genes, which cause activation of the mitogen-activated protein kinase (MAPK)/extracellular signal-regulated kinase (ERK) signaling pathway. PLCH occurs predominantly in young smokers, without gender predominance. Lungs might be involved as an isolated organ or as part of a multiorgan disease. High-resolution computed chest tomography plays an outstanding role in PLCH diagnosis. The typical radiological picture of PLCH is the presence of small intralobular nodules, "tree in bud" opacities, cavitated nodules, and thin- and thick-walled cysts, frequently confluent. Histological examination of the lesion and demonstration of characteristic eosinophilic granulomas with the presence of LCs that display antigen CD1a or CD207 in immunohistochemistry are required for definite diagnosis. Smoking cessation is the most important recommendation for PLCH patients, but treatment of progressive PLCH and multisystem disease is based on chemotherapy. Recently, new targeted therapies have been implemented.
肺朗格汉斯细胞（LC）组织细胞增多症（PLCH）病因不明，是一种罕见的肿瘤性疾病，其特征是骨髓来源的朗格汉斯细胞浸润肺和各种器官，并伴有强烈的炎症反应。这些细胞携带基因和/或基因以及基因的体细胞突变，导致丝裂原活化蛋白激酶（MAPK）/细胞外信号调节激酶（ERK）信号通路激活。PLCH主要发生在年轻吸烟者中，无性别差异。肺部可能作为孤立器官受累，也可能是多器官疾病的一部分。高分辨率胸部计算机断层扫描在PLCH诊断中起着重要作用。PLCH典型的放射学表现为小叶内小结节、“树芽”状阴影、空洞性结节以及薄壁和厚壁囊肿，常相互融合。明确诊断需要对病变进行组织学检查，并在免疫组织化学中显示具有表达抗原CD1a或CD207的LC的特征性嗜酸性肉芽肿。对PLCH患者最重要的建议是戒烟，但对于进行性PLCH和多系统疾病的治疗基于化疗。最近，已实施了新的靶向治疗。
