成人肺朗格汉斯细胞组织细胞增多症五例临床分析

目的 探讨成人肺朗格汉斯细胞组织细胞增多症(PLCH)患者的临床、影像、肺功能和组织病理特点.方法 回顾性分析2006年6月至2007年10月北京朝阳医院收治的5例PLCH患者的临床资料.结果 5例均为男性吸烟者,以肺部损害为主,其中2例以自发性气胸为首发症状,1例为肺脏终末期病变合并肺动脉高压.胸部高分辨率CT示大小不一的多发囊腔阴影,病变以双上肺为著,终末期病例囊腔影遍及全肺.5例均经外科肺活检获得组织病理学资料,显示囊腔样改变和朗格汉斯细胞聚集(免疫组织化学CD1a和S-100染色阳性).肺功能检查示V50占预计值%为53.6%～77.6%,V25占预计值%为38.5%～70.5%,V50和V25的降低与组织病理学所见小气道受累相符合.治疗以戒烟为主,疾病有自然缓解倾向.结论 PLCH是朗格汉斯细胞组织细胞增多症的一种特殊类型,主要发生于吸烟者,多呈良性经过.

Clinical features of adult pulmonary Langerhans'cell histiocytosis

Objective To investigate the clinical features including history, radiology, pulmonary function and histological pathology of adult pulmonary Langerhans' cell histiocytosis ( PLCH ). Method Clinical data of 5 PLCH patients were reviewed from June 2006 to October 2007. Results All 5 patients were smokers with lung involvements. Spontaneous pneumothorax was the initial manifestation in 2 patients. One end-stage patient presented with severe pulmonary arterial hypertension. High-resolution CT scans of the lungs showed multifocal nodules and cysts that predominantly affected the upper and middle lung zones. At microscopic analysis,lung specimen showed discrete bronchiolocentric stellate lesions separated by uninvolved lung with air-space enlargement. Langerhans' cells expressed cell surface CD1 a and cytoplasmic S-100 protein. Cessation of smoking led to stabilization of the disease. The decrease of V50 (53.6% - 77.6% pred) and V25 (38.5% -70. 5% pred) was consistent with small airway damages of lung histology. Conclusion PLCH is an isolated form of Langerhans' cell histiocytosis that primarily affects a single- system in smokers.