肺郎格罕细胞组织细胞增多症7例临床分析

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。

A clinical study of 7 cases of pulmonary Langerhans cell histiocytosis.

Objective To study the clinical features of pulmonary Langerhans cell histiocytosis.Methods Restrospectively review of 7 cases of pulmonary Langerhans cell histiocytosis proved by pathology with the clinical,physical,radiological,pathological and pulmonary function presentation.Results Seven cases were all male,aged from 17 to 37.Four of them are cigarette smokers.The major symptoms were cough and laboring dyspnea,and pneumothorax presented in five cases.As to pulmonary function presentation,obstructive pattern presented in three cases,restrictive pattern and mixed pattern presented in two cases respectively,and the carbon monoxide diffusing capacity was decreased in four cases.The abnormalities present predominantly in the upper and middle lung fields on chest high-resolution CT scan.Lung cysts and reticular changes presented in six cases and nodules presented only in one case.The diagnosis was established by surgical lung biopsy.The positive cases of staining for the S-100,CD1a antigens,and CD68 were 7/7,4/5 and 5/6 respectively.Conclusion PLCH patients are typically young adults,often present with cough,laboring dyspnea and pneumothorax.Pulmonary function presentation is nonspecific and the carbon monoxide diffusing capacity may be decreased.The characteristic features of HRCT are bilateral reticulonodular areas and cysts change that predominantly involved the upper and middle lung zones with relative sparing of the lung bases.The pathologic feathers are interstitial infiltration of bronchiole walls and epithelium by LCH cells and cyst formation.The staining for CD1a antigens and the S-100 proteins is positive by immunohistochemical analysis.