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目的探讨肺郎格罕(Langerhans)细胞组织细胞增多症的临床表现,以提高对本病的认识。方法回顾性分析1997—2006年北京协和医院确诊的7例肺郎格罕细胞组织细胞增多症的临床资料。结果7例患者均为男性,平均年龄26.7岁,其中4例吸烟,主要症状为咳嗽、活动后气短,5例在疾病过程中发生气胸。肺功能示阻塞性通气功能障碍3例、限制性通气功能障碍2例、混合性通气功能障碍2例,4例有不同程度的弥散功能障碍。胸部高分辨CT(HRCT)示6例表现为双上中肺野网格状改变及囊性变,仅有1例可见小结节影。外科肺活检标本病理学检查结果示7例光镜下均可见病理性郎格罕细胞浸润,6例可见囊样及气腔样结构。免疫组化阳性检出情况为7例S-100均为阳性;5例行CD1a检查者中4例阳性;6例行CD68检查者中5例阳性。结论肺郎格罕细胞组织细胞增多症常见于年轻吸烟男性,主要临床表现为咳嗽、活动后气短、反复气胸,胸部HTCT表现为双上中肺野为主的网结节或囊性变,肺功能无特异性改变,可伴有弥散功能异常,病理学检查可见病理性郎格罕细胞或免疫组化CD1a、S-100阳性可以明确诊断。
Objective To investigate the clinical manifestations of Langerhans cell histiocytosis in order to improve the understanding of this disease. Methods The clinical data of 7 cases of lung Langerhans cell histiocytosis confirmed by Peking Union Medical College Hospital during 1997-2006 were retrospectively analyzed. Results All 7 patients were male, with an average age of 26.7 years. Among them, 4 were smokers. The main symptoms were cough, shortness of breath after activity, and pneumothorax in 5 patients during the disease. Pulmonary function showed obstructive ventilatory dysfunction in 3 cases, restrictive ventilatory dysfunction in 2 cases, mixed ventilatory dysfunction in 2 cases, 4 cases with varying degrees of diffuse dysfunction. High-resolution chest CT (HRCT) showed 6 cases of double-on-field lung and mesangial changes of cystic degeneration, only 1 case showed a small nodules. Pathological examination of surgical lung biopsy specimens showed pathological findings of Langerhans cell infiltration in 7 cases under light microscopy, 6 cases of cystic and air-like structures. Immunohistochemistry was detected in 7 cases of S-100 were positive; 5 cases of CD1a in 4 cases positive; 6 cases of CD68 in 5 cases were positive. Conclusions Pulmonary Langerhans cell histiocytosis is common in young smokers. The main clinical manifestations are cough, shortness of breath after exercise and repeated pneumothorax. The chest CT showed nodular or cystic degeneration in the upper middle lung field. No specific changes in function, may be associated with diffuse dysfunction, pathological examination shows that the pathological Langerhans cells or immunohistochemical CD1a, S-100 positive diagnosis can be clear.