Schiaffino E, Tavani E, Dellafiore L, Schmid C
Department of Pathology, San Carlo Borromeo Hospital, Milan, Italy.
Appl Pathol. 1989;7(4):265-72.
Pulmonary lymphangiomyomatosis is a rare disease characterized by wide-spread proliferation of smooth muscle cells around lymphatic and blood vessels, smaller airways and in the interstitium. Young women in the fertile status are almost exclusively affected. Spontaneous recurrent pneumothorax is the most important symptom. Chylous effusions are reported in 37.5% of cases. Our observation deals with a woman aged 43. Light- and electron-microscopic findings are described. Estrogen receptors have been detected on smooth muscle cells. Bilateral oophorectomy and treatment with progesterone were successful.
肺淋巴管平滑肌瘤病是一种罕见疾病，其特征是淋巴管、血管周围、较小气道及间质中平滑肌细胞广泛增殖。几乎仅累及处于生育期的年轻女性。自发性反复气胸是最重要的症状。37.5%的病例报告有乳糜性胸腔积液。我们观察的是一位43岁女性。描述了光镜和电镜检查结果。已在平滑肌细胞上检测到雌激素受体。双侧卵巢切除术及孕激素治疗取得了成功。
