实用临床医药杂志2012，Vol.16Issue(17)：158-160,3.
肺淋巴管平滑肌瘤病的临床病理特征及影像学表现
Clinical and pathological features and imaging manifestations of pulmonary lymphangioleiomyomatosis
摘要
Abstract
Objective To investigate the clinical and pathological features and imaging manifestations of pulmonary lymphangioleiomyomatosis (PLAM). Methods Two PLAM patients were observed in terms of clinical features, imaging manifestations and pathological features. Results The clinical manifestations of PLAM were progressive shortness of breath ( dyspnea ) , recurrent spontaneous pneumothorax, chylotborax and haemopthsis. PLAM carried characteristic high-resolution CT (HRCT) sings, which showed multiple thin-walled cysts distributed evenly throughout the bilateral lung fields. Pathologic examination of lung biopsy specimens showed abnormal, perivascular proliferation of smooth muscle in and around the small airway, lymphatic and small blood vessels. Immunophenotypic findings revealed positive staining for HMB-45, SMA, but negative for ER and PR. Conclusion PLAM is a rare neoplastic proliferative disease in the lung. Pathologic examination of lung tissue biopsy is required for confirmation of PLAM diagnosis.关键词
淋巴管平滑肌瘤病/肺/病理Key words
lymphangioleiomyomatosis/ pulmonary/ pathology分类
医药卫生引用本文复制引用
韩锋锋,杨天芸,郏琴,李惠民,管雯斌,郭雪君..肺淋巴管平滑肌瘤病的临床病理特征及影像学表现[J].实用临床医药杂志,2012,16(17):158-160,3.基金项目
中国高校医学期刊临床专项资金(11220138) （11220138）
