Tsai Chiao-Yun, Huang Hsu-Chih, Xu Zhen-Dong, Hsia Jiun-Yi, Chen Chih-Yi
Division of Thoracic Surgery, Department of Surgery Chung Shan Medical University Hospital Taichung Taiwan, Republic of China.
Institute of Medicine Chung Shan Medical University Taichung Taiwan, Republic of China.
Respirol Case Rep. 2022 Mar 27;10(4):e0939. doi: 10.1002/rcr2.939. eCollection 2022 Apr.
Pulmonary Langerhans cell histiocytosis is a rare disease caused by the proliferation of CD1a-positive histiocyte-like cells infiltrating the lung's interstitial layer. Most cases affect young to middle-aged persons, especially adult heavy cigarette smokers. A 49-year-old male heavy smoker (40 pack-year), with non-productive cough, dyspnoea and desaturation, presented with a right-sided pneumothorax on chest x-ray with total atelectasis. Chest computed tomography (CT) revealed bilateral multiple thick-walled infiltrated cysts and multiple ground-glass nodules throughout the entire lung. Surgery with minimal invasive thoracoscopic lung biopsy and pleurodesis was performed. Pathology showed histiocyte-like cells aggregates in the pulmonary parenchyma. Immunohistochemical stain demonstrated CD1a(+), S100(+) and CD68(+). After 3 months of smoking cessation, clear improvement was evidenced with a chest CT showing bilateral multiple thin-walled rounded cysts and multiple ground-glass nodules that are smaller in size and decreased in numbers. Early minimal invasive thoracoscopic lung biopsy and pleurodesis can also be a choice if the development of secondary spontaneous pneumothorax occurs.
肺朗格汉斯细胞组织细胞增多症是一种罕见疾病，由浸润肺间质层的CD1a阳性组织细胞样细胞增殖引起。大多数病例累及青年至中年人群，尤其是成年重度吸烟者。一名49岁重度吸烟男性（吸烟史40包年），有干咳、呼吸困难和低氧血症，胸部X线显示右侧气胸伴完全肺不张。胸部计算机断层扫描（CT）显示双侧全肺多发厚壁浸润性囊肿和多发磨玻璃结节。行微创胸腔镜肺活检及胸膜固定术。病理显示肺实质内有组织细胞样细胞聚集。免疫组化染色显示CD1a(+)、S100(+)和CD68(+)。戒烟3个月后，胸部CT显示明显改善，双侧出现多个薄壁圆形囊肿和多个尺寸更小、数量减少的磨玻璃结节。如果发生继发性自发性气胸，早期微创胸腔镜肺活检及胸膜固定术也可作为一种选择。
