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目的:探讨肺淋巴管平滑肌瘤病患者的临床、影像学表现及病理改变特征。方法:回顾性分析呼吸科近年诊治的2例肺淋巴管平滑肌瘤病患者的临床、影像学资料,并复习相关文献。结果:患者主要为女性,临床表现为进行性呼吸困难、反复发作的气胸等;胸部CT主要表现为双肺弥漫分布多发的囊样改变;病理改变主要为异常增殖的平滑肌样细胞,破坏肺实质。肺淋巴管瘤病临床症状缺乏特异性常易误诊,诊断需结合病史、影像学以及典型病理改变。肺移植是唯一有效的治疗手段。结论:育龄期女性,反复发作气胸、肺部CT表现为弥漫性囊样改变时,应警惕肺淋巴管平滑肌瘤病可能,必要时需行病理学检查以明确诊断。
Objective: To investigate the clinical manifestations and pathological features of patients with pulmonary lymphangioleiomyomatosis. Methods: The clinical and imaging data of 2 patients with pulmonary lymphangioleiomyomatosis who were diagnosed and treated in recent years in respiratory department were analyzed retrospectively. The related literatures were reviewed. Results: The patients were mainly female, with clinical manifestations of progressive dyspnea and recurrent pneumothorax. Chest CT mainly manifested as cystic changes of multiple pulmonary diffuse distribution. The main pathological changes were abnormal proliferation of smooth muscle-like cells, destruction of lung parenchyma . The clinical manifestations of pulmonary lymphangiomatosis lack of specificity often misdiagnosis, diagnosis should be combined with medical history, imaging and typical pathological changes. Lung transplantation is the only effective treatment. Conclusion: Women of reproductive age, recurrent pneumothorax, pulmonary CT manifestations of diffuse cystic changes should be alert to pulmonary lymphangioleiomyomatosis may need pathological examination to confirm the diagnosis if necessary.