肺淋巴管平滑肌瘤病临床病理学观察

Clinicopathologic analysis of pulmonary lymphangioleiomyomatosis

目的 探讨肺淋巴管平滑肌瘤病临床、病理特征。方法 对5例肺淋巴管平滑肌瘤病临床资料进行收集分析,HE切片观察,采用免疫组织化学(SP法)检测平滑肌肌动蛋白(SMA)、HMB45、基质金属蛋白酶(MMP)2、孕激素受体(PR)、雌激素受体(ER),并进行文献复习。结果 肺淋巴管平滑肌瘤病是原因不明的肺部疾病,只发生在女性,特别是绝经前妇女。临床表现为呼吸困难,咯血,气胸和乳糜胸等。病理学检查显示不同成熟度平滑肌细胞在细支气管壁、肺泡壁、淋巴管壁和血管壁周围增生,肺实质呈囊性变。增生的平滑肌细胞免疫组织化学5例SMA、HMB45、MMP2均阳性; 1例的ER和PR均阳性, 1例仅ER阳性, 1例仅PR阳性, 1例的ER和PR均阴性。结论 育龄期妇女如反复出现自发性气胸、咯血、活动后呼吸困难应考虑肺淋巴管平滑肌瘤病的可能,病理检查可确定肺淋巴管平滑肌瘤病的诊断。

Objective To investigate the clinicopathological features of pulmonary lymphangioleiomyomatosis (PLAM). Methods By means of HE and immunohistochemistry (SP method) studies, the clinical and pathological features of 5 PLAM cases were analyzed and the related literature reviewed. Results PLAM was a rare lung disease of unknown etiology and was restricted to females who were generally pre-menopausal. Pathological features showed abnormal smooth muscle cells(LAM cells) line the airways, lymphatics and blood vesssels leading to airflow obstruction and replacement of the lung parenchyma by cysts.LAM cells were positive for HMB45. Clinically the disease was categorized by dyspnoea, haemoptysis, recurrent pneumothoraces and chylous effusions. Conclusions PLAM should be considered when recurrent pneumothorax, haemoptysis and dyspnoea occur in females. Pathologic examination of lung tissue biopsy is required for confirmation of PLAM diagnosis.