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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 clinical and pathological features of pulmonary lymphatic leiomyoma (PLAM) and to improve its understanding of the disease. Methods: One case of PLAM patients with clinical features, HE and immunohistochemical staining results were analyzed, combined with the literature review. RESULTS: PLAM was a persistent diffuse lung disease that occurred almost exclusively in women of childbearing age. The main clinical symptoms were progressive dyspnea, repeated pneumothorax and chylothorax. Pathological features show that immature smooth muscle-like cells proliferate around the bronchiolar wall, alveolar wall, lymphatic wall, and vessel wall. Immunohistochemical staining showed that the expression of Desmin, SMA, Vimentin and HMB45 in PLAM cells were positive, and some cells were positive for ER and PR. CONCLUSIONS: PLAM has characteristic features in imaging and histopathology. Immunohistochemistry HMB-45 positivity is specific. Due to extensive lung disease, the prognosis is poor. There has been some progress in the study of the pathogenesis and treatment of PLAM, but more research is needed in the future.