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目的探讨肺郎格罕细胞组织细胞增多症(pulmonary Langerhans cell histiocytosis,PLCH)的临床特征、诊断和治疗。方法回顾性分析1999年2008年4例病理确诊的PLCH。结果 4例患者均为男性;13～56岁;2例吸烟。临床特征是活动后气短、气胸。胸部CT表现为网格状、囊状或结节状影像。肺活检病理结果 :光学显微镜下可见病理性郎格罕细胞,免疫组织化学法检测发现4例S-100均为阳性,2例CD1a阳性,1例CD68阳性。结论 PLCH胸部CT表现为网结节或囊性变,病理检查见病理性郎格罕细胞浸润细支气管壁和上皮细胞的间质,免疫组织化学CD1a抗原、S-100蛋白阳性可明确诊断。
Objective To investigate the clinical features, diagnosis and treatment of pulmonary Langerhans cell histiocytosis (PLCH). Methods Retrospective analysis of 4 cases of pathologically diagnosed PLCH in 1999. Results All 4 patients were male, 13 to 56 years old and 2 were smoking. Clinical features are shortness of breath after exercise, pneumothorax. Chest CT showed a grid-like, cystic or nodular image. Pathological results of lung biopsy: Pathological Langerhan cells were observed under optical microscope. Immunohistochemistry showed that all four S-100 cells were positive, two were CD1a positive and one was CD68 positive. Conclusion PLCH chest CT showed cystic nodules or cystic degeneration. Pathological examination revealed the interstitial infiltration of the bronchiole wall and epithelial cells of pathological Langerhans cells. Immunohistochemistry CD1a antigen and S-100 protein positive could be diagnosed.