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目的探讨肺朗格汉斯细胞组织细胞增生症(PLCH)的临床特征,提高对该疾病的认识。方法回顾性分析2004年8月-2013年9月经病理组织学检查确诊为PLCH的4例患者的临床资料,并对部分患者进行随访分析。结果 4例患者均为男性,年龄19~46岁,3例吸烟。主要症状为胸闷、咳嗽、呼吸困难。2例在疾病过程中发生气胸,1例合并肺结核。胸部CT主要表现为双上中肺为主的囊状、结节状、网格状影。4例病变组织在光学显微镜下均可见病理性朗格汉斯细胞浸润。4例免疫组织化学染色CD1a及S100均阳性,其中2例Langrin染色阳性(另外2例未行该染色)。4例患者中2例口服激素治疗,1例症状改善。合并肺结核的患者经抗结核治疗后症状改善,CT图像上实变病灶部分吸收。3例患者失访。结论 PLCH主要见于男性吸烟患者,主要表现为胸闷、咳嗽、活动后呼吸困难、反复气胸。胸部高分辨率CT表现为双上中肺为主的囊状、结节状、网状影。病灶部位病理组织学检查见病理性朗格汉斯细胞,或免疫组织化学染色CD1a/Langerin阳性可明确诊断。
Objective To investigate the clinical features of lung Langerhans cell histiocytosis (PLCH) and to improve the understanding of the disease. Methods The clinical data of 4 patients diagnosed as PLCH by histopathology from August 2004 to September 2013 were analyzed retrospectively. Some patients were followed up. Results All 4 patients were male, aged 19 to 46 years and 3 smoked. The main symptoms are chest tightness, cough, difficulty breathing. Two patients experienced pneumothorax during the disease and one patient had pulmonary tuberculosis. Chest CT mainly for the double in the lung-based cystic, nodular, grid-like shadow. Pathological Langerhans cell infiltration was observed in 4 cases of diseased tissue under optical microscope. Four cases of immunohistochemical staining CD1a and S100 were positive, two cases of positive Langrin staining (the other two did not do the staining). Two of the four patients received oral hormonal therapy and one of the patients improved symptoms. In patients with tuberculosis, the symptoms improved after anti-TB treatment, and the solidified lesions on the CT images were partially absorbed. Three patients were lost. Conclusion PLCH mainly found in male smoking patients, mainly manifested as chest tightness, cough, dyspnea after activity, repeated pneumothorax. High-resolution chest CT showed double lung-based cystic, nodular, reticular shadow. Histopathological examination of the lesion pathological Langerhans cells, or immunohistochemical staining CD1a / Langerin positive can be a clear diagnosis.