肺朗格罕氏细胞组织细胞增多症的影像学诊断

Imaging diagnosis of pulmonary Langerhans'cell histiocytosis

目的探讨肺郎格罕氏细胞组织细胞增生症(PLCH)的影像学表现及鉴别诊断。方法6例经病理证实的PLCH患者均经胸部X线平片和高分辨率CT(HRCT)检查,对其影像学表现进行了回顾性分析,并结合文献复习讨论了鉴别诊断。结果X线胸片与HRCT显示了双肺弥漫网状、星状结节影2例,弥漫性分布的囊疱与星状结节并存2例,两肺实变影、磨玻璃样影是1例,以及单侧肺斑片状浸润性改变1例。结论两肺弥漫性分布的网状、星状结节影及囊疱影表现结合临床及实验室检查可提示PLCH的诊断。PLCH应与肺淋巴管平滑肌瘤病、特发性间质纤维化,以及结节病等鉴别。

Objective To discuss the imaging findings and differentical diagnoses of pulmonary Langerhans＇ s cell histiocytosis （PLCH） . Methods Six patients with pathologically proved PLCH underwent chest X- ray radiographies and HRCT scans. The imaging findings in all patients were retrospectively analyzed and then the differential diagnosis was discussed together with review of the literature. Results Among 6 patients with PLCH, X - ray plain films and CT scans showed diffuse - reticular or satellate nodular shadows in both lungs（ n = 2）, the coexistence of diffuse- distributed cystic blebs and satellate- nodular shadows in both lungs（ n = 2）, consolidation and ground glass shadows in both lungs（ n = 1 ）, and patchy - infiltrating changes in one lung（ n = 1 ）. Conclusion Overally analyzing the imaging findings of diffuse distributed reticular, satellate and cystic bleb shadows in both lungs combined with clinical and laboratory examinations can suggest the diagnosis of PLCH. This disease should be differentiated from lymphangia leiomyoma of the lung, idiopathic interstitial fibrosis and sarcoidosis. [Key words]