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华西医学  2015 

肺朗格汉斯细胞组织细胞增生症临床分析及文献复习

DOI: 10.7507/1002-0179.20150575, PP. 2013-2018

Keywords: 肺朗格汉斯细胞组织细胞增生症,临床特征,高分辨率CT

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Abstract:

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。

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