肺淋巴管平滑肌瘤病临床病理分析及文献复习

Pulmonary Lmphangioleiomyomatosis:a Cinicpathological Aalysis and Review of Literature

目的:探讨肺淋巴管平滑肌瘤病(PLAM)的临床和病理特征,提高对该病的认识。方法:对1例PLAM患者的临床特点、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,几乎发生于育龄期妇女,主要临床症状为进行性呼吸困难、反复气胸及乳糜胸。病理特征显示未成熟平滑肌样细胞在细支气管壁、肺泡壁、淋巴管壁和血管壁周围增生。免疫组化染色显示增生PLAM细胞Desmin、SMA、Vimentin、HMB45均呈阳性表达,部分细胞ER及PR阳性。结论: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。由于肺部广泛病变,预后较差。对PLAM的发病机制和治疗的研究已有了一定的进展,但今后尚需更深入地研究。

Objective： To investigate the clinicopathological features of pulmonary lymphangioleiomyomatosis （PLAM） and enhance under standing. Methods： A PLAM case was analyzed by HE and immuno histochemical staining, and the related literature reviewed. Results： PLAM was a rare diffuse interstitial plumonary disease and was almost restricted to females who were generally premenopausal. The main clinicalmanifestations included dysnpea, pneumothorax and chylothorax. Pathological hallmarks of LAM are diffuse infiltration of the pulmonary parenehyma with atypical smooth muscle cells, include bronchioles, lymphatics and blood vesssels. IHC showed that characteristic smooth muscle cells were positive for Desmin, SMA, Vimentin, HMB45, estrogen and progesterone receptor expression. Conclusions： PALM has characteristic manifestation of clinicopathology and radiology, IHC show positive of HMB-45. As a diffuse lesion, the prognosis is worse. Now the research about the diagnosis and treatment of PLAM has improved, but we must investigate it more deeply in the future.