Keane Ruaidhri J, Subramaniam Abirami, Varghese Chithra, Jeffers Michael, Donnelly Seamas C
Department of Respiratory Medicine, Medical Professorial Unit, Tallaght University Hospital & Trinity College, Dublin, Ireland.
Department of Cellular Pathology, Tallaght University Hospital, Ireland.
Respir Med Case Rep. 2020 Nov 7;31:101280. doi: 10.1016/j.rmcr.2020.101280. eCollection 2020.
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare cystic lung disease. The natural history is often unpredictable making it difficult to diagnose. We report a 63-year-old male with dyspnoea, chronic cough and recurrent respiratory tract infections, who developed progressive multifocal cystic lesions on pulmonary nodule surveillance over 4 years. He was a heavy smoker with a history of multiple spontaneous pneumothoraces in his teens. Extensive investigations culminated in a thoracoscopic wedge resection, which identified histiocytic nodules staining positive for CD1a and thus confirming the diagnosis of PLCH. It is now apparent that PLCH was the likely cause of his pneumothoraces.
肺朗格汉斯细胞组织细胞增多症（PLCH）是一种罕见的囊性肺病。其自然病史往往不可预测，难以诊断。我们报告一名63岁男性，有呼吸困难、慢性咳嗽和反复呼吸道感染症状，在4年的肺部结节监测中出现了进行性多灶性囊性病变。他是重度吸烟者，十几岁时曾多次发生自发性气胸。经过广泛检查，最终进行了胸腔镜楔形切除术，发现组织细胞性结节CD1a染色呈阳性，从而确诊为PLCH。现在很明显，PLCH可能是他气胸的病因。
