Clementsen P S, Folke K, Faurschou P
Medicinsk-lungemedicinsk afdeling Y, Amtssygehuset i Gentofte.
Ugeskr Laeger. 1995 Jan 16;157(3):298-9.
Lymphangioleiomyomatosis (LAM) is a rare disease, which only affects women of childbearing age. A case of LAM in a 36-year-old woman is presented. Patho-anatomical findings are smooth muscle hyperplasia leading to obstruction of lymphatics (possibly resulting in chylothorax), blood vessels (possibly resulting in haemoptysis) and bronchi (possibly resulting in emphysema and pneumothorax). High resolution computed tomography (HRCT) of the chest can show the typical multiple small cysts and bullous emphysema distributed diffusely throughout both lungs together with interstitial fibrosis. Treatment of the disease aims at an inhibition of the presumed growth-promoting effect of oestrogen on the smooth muscle cells. However, progression is often seen in spite of treatment.
淋巴管平滑肌瘤病（LAM）是一种罕见疾病，仅影响育龄女性。本文报告一例36岁女性的LAM病例。病理解剖学发现为平滑肌增生，导致淋巴管阻塞（可能导致乳糜胸）、血管阻塞（可能导致咯血）和支气管阻塞（可能导致肺气肿和气胸）。胸部高分辨率计算机断层扫描（HRCT）可显示典型的多发小囊肿和大疱性肺气肿，弥漫分布于双肺，并伴有间质纤维化。该疾病的治疗旨在抑制雌激素对平滑肌细胞的假定促生长作用。然而，尽管进行了治疗，病情仍常出现进展。
