肺淋巴管平滑肌瘤病临床病理学特征

Clinicopathological features of pulmonary lymphangioleiomyomatosis:a report of 3 cases

目的探讨肺淋巴管平滑肌瘤病(pulmonarylymphangioleiomyomatosis,PLAM)临床和病理特征。方法对3例PLAM患者的临床资料、HE及免疫组化染色结果进行分析,并结合文献进行复习。结果PLAM是一种持续发展的弥漫性肺疾病。只发生在女性,特别是绝经前妇女。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。高分辨率CT(HRCT)可见两肺弥漫性分布的薄壁小囊状改变。组织学特点为肺淋巴管、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。免疫组化结果显示3例均表达HMB-45、SMA、actin、MMP-2、desmin,2例表达PR、ER。经随访,有2例死亡。结论育龄期妇女发生渐进性呼吸困难,并反复出现气胸,胸部HRCT示两肺弥漫性分布薄壁囊状改变,临床上应考虑到PLAM的可能,最好能行肺组织活检明确诊断。PLAM在影像学与病理组织学上有特征性表现,免疫组化HMB-45阳性具特异性,预后较差。

Purpose To investigate the clinicopathological features of pulmonary lymphangioleiomyomatosis （PLAM）. Methods Three PLAM cases were analyzed by HE and immunohistochemical staining, and the related literature reviewed. Results PLAM was a rare diffuse interstitial plumonary disease of unknown cause and was restricted to females who were generally premenopausal. PLAM was characterized by recurrent spontaneous pneumothorax shortness of breath （dyspnea） with physical activity, haemopthsis and chylothorax. High resolution CT scan （HRCT） showed multiple thin walled cysts distributed evenly throughout the bilateral lung fields with normal intervening lung parenchyma. Pathological characteristics were abnormal, perivascular proliferation of smooth muscle in and around the small airway, lymphatic and small blood vessels. Immunohistochemical test showed HMB-45, SMA, actin, MMP-2, desmin were positive in all cases, two of which expressed ER and PR. Two cases died during follow-up. Conclusions It should be considered the probability of PLAM when women of childbearing ages have some clinical manifestations as follows, the increasing dyspnoea that can not be relieved similar to emphysema, HRCT shows multiple thin walled cysts distributed evenly throughout the bilateral lung fields with normal intervening lung parenchyma, and recurrent pneumothorax or chylothorax. The optimal confirm diagnostic method is lung biopsy.