单独肺受累的儿童肺郎格罕细胞组织细胞增生症一例报道并文献分析

目的 了解单独肺受累的儿童肺郎格罕细胞组织细胞增多症(PLCH)的临床和实验室特点.方法 回顾性分析南京儿童医院确诊的1例单独肺受累的PLCH的临床资料,并结合文献报道的11例单独肺受累的PLCH进行分析.结果 本组患儿为2岁11个月男童,病程仅1个月,临床表现为咳嗽、活动后气短,胸部CT显示双肺弥漫分布点状、结节状、网状及条索状高密度影.开胸肺活检免疫组化提示病变组织表达S-100散在(+),CD1α,CD68灶性(+),无其他器官系统病变,经泼尼松、VP16、长春地辛化疗后临床症状基本消失,胸部CT病变明显好转.文献报道的11例中起病症状为咳嗽、呼吸困难者10例；胸部CT(8例)表现分别显示间质性病变(5例)、囊样病变(5例)、结节样影(2例)、气胸(2例)；9例肺活检病变组织找到郎格罕细胞浸润,部分行免疫组化提示表达CD1α,另2例肺泡灌洗液细胞免疫组化提示表达CD1α及S-100.结论 儿童单独肺受累PLCH临床上以咳嗽、呼吸困难为主要表现,影像学上常见间质性病变、小结节影或囊样病变.肺活检组织常可见郎格罕细胞浸润,病变组织及肺泡灌洗液细胞的免疫组化表达CD1α.

Isolated nulmonarv Langerhans cell histiocvtosis in a two-year-old child: case report and literature review

Objective To improve the recognition of the clinical features and results of laboratory examination for isolated pulmonary Langerhans cell histiocytosis (PLCH) in children. Method The information of one case with isolated PLCH was analyzed and reports of 11 cases with isolated PLCH were reviewed.Result The patient we report is only 2 years old with 1 month of course of disease,manifesting with prominent pulmonary involvement: cough and short of breath; CT scan of the chest showed punctiform,nodular and reticular high density opasities involving all lobes of both lungs.Biopsy of the lung tissue showed expression of CD1a,CD68,S-100,consistent with the diagnosis of LCH.He received prednisolone,VP16and Vindesine with good response.Ten of 11 cases of isolated PLCH reported before manifesting with cough and dyspnea,CT scan of the chest showed interstitial lung changes (5/8),cystic changes (5/8),small nodules (2/8) and pneumothorax (2/8).Langerhans cells were found in 9 cases on lung biopsy,part of biopsy lung tissues were stained with anti-CDla,the alveolar lavage fluid of the other 2 cases were stained with S-100 and anti-CD1a.Conclusion Isolated PLCH is rarely reported in children.It manifested with prominent pulmonary involvement: cough and short of breath,and CT scan of the chest showed interstitial lung changes,small nodules or cysts involving the lung,Langerhans cell could be found in lung biopsy,and the immunohistochemical staining in lung biopsy lung and alveolar lavage fluid stained with S-100 and antiCDla antibodies.