Wissler U, Siebenmann R, Lupi G A, Kistler H J
Schweiz Med Wochenschr. 1976 Jan 17;106(3):84-93.
Diffuse lymphangiomyomatosis is a rare disorder of the mediastinal and retroperitoneal lymph nodes due to chronic benign proliferation of the smooth muscle cells in the lymphatics. This process leads to chylothorax and chylascites and eventually to cystic lung disease. The pulmonary complications are usually result in respiratory failure and death. The disease is only observed in females. A typical lethal course in a young woman is described and the literature is reviewed. The possible etiologic and therapeutic implications are discussed.
弥漫性淋巴管肌瘤病是一种罕见的疾病，由淋巴管中平滑肌细胞的慢性良性增殖引起，累及纵隔和腹膜后淋巴结。这一过程导致乳糜胸和乳糜腹水，最终发展为囊性肺疾病。肺部并发症通常导致呼吸衰竭和死亡。该疾病仅在女性中观察到。本文描述了一名年轻女性的典型致命病程并对文献进行了综述。同时讨论了可能的病因及治疗意义。
