Villegas Fernández F R, Callol Sánchez L, Moreno Muro M, Coca Menchero S, García Pérez C, Gómez de Terreros F J
Servicios de Neumología y de Anatomía Patológica, Hospital del Aire, Madrid.
Rev Clin Esp. 1992 Jul;191(3):144-7.
The case concerned is one of lymphangiomyomatosis (LAM) in a woman aged 39, that after a episode of spontaneous pneumothorax was subject to medical research for progressive dyspnea of medium efforts and bloodstained sputum. High resolution CT showed numerous thin-walled cystic airspaces of varying sizes distributed diffusely throughout the lungs. The abdominal CT and ultrasound developed multiple right renal angiomyolipomas. The histopathological study of the sample obtained by open lung biopsy was conclusive of LAM. The main feature of this rare disease, which occurs almost exclusively in women of reproductive age, is the abnormal proliferation of immature smooth muscle at the level of the distal airway, small blood vessels and lung lymphatic system, including sometimes the mediastinal and retroperitoneal lymphatic system. The clinical, functional, radiological and differential diagnostic aspects with entities histopathologically similarities are discussed and the therapeutic possibilities are reviewed.
该病例是一名39岁女性的淋巴管平滑肌瘤病（LAM），在一次自发性气胸发作后，因中度用力时进行性呼吸困难和血痰接受医学研究。高分辨率CT显示，肺部弥漫分布着许多大小不一的薄壁囊状气腔。腹部CT和超声检查发现右肾有多个血管平滑肌脂肪瘤。经开放式肺活检获取的样本进行组织病理学研究确诊为LAM。这种罕见疾病几乎仅发生于育龄女性，其主要特征是远端气道、小血管和肺淋巴系统（有时包括纵隔和腹膜后淋巴系统）水平的未成熟平滑肌异常增殖。文中讨论了其临床、功能、放射学及与组织病理学相似实体的鉴别诊断方面，并综述了治疗可能性。
