基于肺活检的原发性肺朗格汉斯细胞组织细胞增生症病理分型及预后分析

Pulmonary Langerhans's cell histocytosis based on tissue biopsy emphasis:A comprehensive analysis of pathological classification and prognosis

目的:总结肺朗格汉斯细胞组织细胞增生症(pulmonary Langerhans’cell histiocytosis,PLCH)临床病理特征及预后资料,提高对该病的诊断率,减少不良预后。方法:回顾性分析2002年至2019年间南京某医院经病理确诊的PLCH 7例,讨论其组织起源、临床特征、病理特点及预后。结果:7例患者年龄18~61岁(平均33岁),男∶女为5∶2。6例以咳嗽、胸闷为首发症状,1例表现为腰背部疼痛,体检发现双肺肺大泡就诊。胸部CT 3例示两肺弥漫性病变,4例示单侧气胸改变。组织学见团块状聚集的朗格汉斯细胞,伴不等量的嗜酸性粒细胞、淋巴细胞、组织细胞及中性粒细胞浸润,周围肺组织可见纤维组织增生及肺气肿、肺大泡形成。免疫组化染色朗格汉斯细胞表达Langerin、S-100、CD1a。本组病例多数(4/7例)无吸烟史。随访6例原发性PLCH均存活,病情平稳无进展;1例多系统PLCH(累及肺、骨骼),18个月后死于呼吸衰竭。结论:PLCH罕见,中青年男性患者居多,以呼吸道症状起病,特别是非吸烟患者胸部影像学检查提示气胸或弥漫性病变时,应警惕PLCH的发生可能。胸腔镜下肺活检或部分肺叶切除送病检可明确诊断。老年人、肺部弥漫性结节性病变及多系统PLCH患者预后差。

Objective:To summarize the clinicopathological features and prognosis of pulmonary Langerhans'cell histiocytosis(PLCH),improve the rate of diagnosis and reduce the adverse prognosis.Methods:7 cases of PLCH confirmed by pathology at the hospital in Nanjing in 2002 to 2019 years were retrospectively analysed.The origin,clinical features,pathological characteristics and prognosis of the tissue were discussed.Results:The age of 7 patients ranged from 18 to 61 years(average 33 years).The ratio of male/female was 5/2.Cough and chest tightness were the first symptoms in 6 patients.One patient was diagnosed with double lung bullae due to back pain.3 cases of thoracic CT showed diffuse lesions of two lungs,and 4 cases showed a change of unilateral pneumothorax.Histological cluster of Langerhans cells were accompanied by an unequal amount of eosinophilic granulocytes,lymphocytes,tissue cells and neutrophils infiltration.Surrounding lung tissue showed fibrous tissue hyperplasia and emphysema,lung vesicles formation.Immunohistochemical staining of Langerhans cells expressed Langerin,S-100 and CD1a.Most of the cases(4/7)had no smoking history.Follow-up of 6 cases of primary PLCH showed survival,stable condition and no progress.One case of multisystem PLCH(involving lung and bone)died of respiratory failure 18 months later.Conclusion:PLCH is rare.Clinical symptoms lack specificity.Misdiagnosis and missed diagnosis were common.For young and middle-aged patients with respiratory symptoms and no smoking,when chest imaging examination indicates pneumothorax or diffuse lesions,PLCH should be vigilant.Thoracoscopic lung biopsy or partial pneumonectomy followed by pathological examination can make a definite diagnosis.The prognosis of elderly patients with diffuse pulmonary nodules and multiple systems is poor.