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目的提高对肺淋巴管平滑肌瘤病(PLAM)的认识。方法对1例PLAM患者的临床资料进行分析,并结合文献进行复习。结果PLAM是一种持续发展的弥漫性肺间质疾病。临床表现为反复发作的自发性气胸、活动后呼吸困难、咯血和乳糜胸等。胸部X线表现两肺弥漫性网格状,晚期呈蜂窝样变化,高分辨力CT(HRCT)可见两肺弥漫性分布的薄壁小囊状改变,病理学特点为肺淋巴管、小气道、小血管的管壁及其周围的平滑肌细胞弥漫性异常增生。免疫组化HMB45阳性。结论育龄期妇女发生渐进性呼吸困难,类似肺气肿的临床表现,并反复出现气胸或乳糜胸水,胸部HRCT示两肺弥漫性分布薄壁囊状改变,临床上应想到PLAM的可能,最好能行肺组织活检明确诊断。
Objective To improve the understanding of pulmonary lymphatic leiomyoma (PLAM). Methods The clinical data of 1 patient with PLAM were analyzed and reviewed with the literature. Results PLAM is a persistent and diffuse interstitial lung disease. Clinical manifestations of recurrent spontaneous pneumothorax, dyspnea after activity, hemoptysis and chylothorax and so on. Chest X-ray showed diffuse grids of both lungs and late-stage cellular changes. High-resolution CT (HRCT) showed diffuse distribution of thin-walled cystic lesions in both lungs. The pathological features were pulmonary lymphatic vessels, small airways, Diffuse dysplasia of smooth muscle cells around the walls of small vessels and around them. Immunohistochemistry HMB45 positive. Conclusions Women of childbearing age have progressive dyspnea, which is similar to the clinical manifestations of emphysema. The recurrence of pneumothorax or chylous pleural effusion, chest CT HRCT showed diffuse distribution of thin-walled cystic changes, the clinical should think of the possibility of PLAM, the best Able to do a clear diagnosis of lung biopsy.