[成人肺朗格汉斯细胞组织细胞增多症的临床特征]

[Clinical features of adult pulmonary Langerhans' cell histiocytosis].

作者信息

Ye Qiao, Dai Hua-Ping, Li Xue, Zhang Lei, Li Hui, Wang Chen

机构信息

Beijing Institute of Respiratory Medicine, Department of Respirology Medicine, Beijing Chaoyang Hospital, Capital Medical University, Beijing 100020, China.

出版信息

Zhonghua Jie He He Hu Xi Za Zhi. 2008 Jul;31(7):492-6.

PMID:19035226

Abstract

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 CD1a 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.

摘要

目的

探讨成人肺朗格汉斯细胞组织细胞增多症（PLCH）的临床特征，包括病史、影像学、肺功能及组织病理学。

方法

回顾2006年6月至2007年10月间5例PLCH患者的临床资料。

结果

所有5例患者均为吸烟者且有肺部受累。2例患者以自发性气胸为首发表现。1例终末期患者表现为严重肺动脉高压。肺部高分辨率CT扫描显示多灶性结节和囊肿，主要累及肺上叶和中叶。显微镜分析显示，肺标本可见散在的以细支气管为中心的星状病变，周围为未受累肺组织，气腔扩大。朗格汉斯细胞表达细胞表面CD1a和细胞质S-100蛋白。戒烟可使病情稳定。V50（预计值的53.6%-77.6%）和V25（预计值的38.5%-70.5%）的降低与肺组织学上的小气道损伤一致。