Takahashi K, Takahashi H, Maeda K, Homma S, Uekusa T, Dambara T, Kira S
Dept of Respiratory Medicine, Juntendo University School of Medicine, Tokyo, Japan.
Eur Respir J. 1995 Oct;8(10):1799-802. doi: 10.1183/09031936.95.08101799.
Pulmonary lymphangiomatosis is a rare clinical and pathological entity which is distinct from lymphangiomyomatosis and from pulmonary lymphangiectasis. We report a case of a 20 year old man with diffuse lymphangiomatosis involving the mediastinum, lungs and retroperitoneum. The patient's intrathoracic lymphangiomatosis produced restrictive and obstructive impairment of his pulmonary function, but did not result in chylothorax or chylopericardium, which are two common manifestations of the disease. The patient's clinical course was remarkable for the concomitant development of chronic disseminated intravascular coagulation (DIC). Lymphangiomatosis involving both the mediastinum and pulmonary parenchyma is rare, and DIC is a very unusual complication of lymphangiomatosis.
肺淋巴管瘤病是一种罕见的临床和病理实体，有别于淋巴管平滑肌瘤病和肺淋巴管扩张症。我们报告一例20岁男性，患有累及纵隔、肺和腹膜后的弥漫性淋巴管瘤病。患者的胸内淋巴管瘤病导致其肺功能出现限制性和阻塞性损害，但未导致乳糜胸或乳糜心包，而这是该疾病的两种常见表现。患者的临床病程因同时并发慢性弥散性血管内凝血（DIC）而引人注目。累及纵隔和肺实质的淋巴管瘤病较为罕见，而DIC是淋巴管瘤病一种非常不寻常的并发症。
