[Lymphangiomyomatosis. Presentation of a case and review of the literature].
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.
D000328:Adult; D005260:Female; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D008206:Lymphatic Diseases; D008477:Mediastinal Diseases; D009130:Muscle, Smooth / Q000648:ultrastructure; D010996:Pleural Effusion / Q000473:pathology; D011030:Pneumothorax / Q000209:etiology; D011654:Pulmonary Edema / Q000209:etiology; D012131:Respiratory Insufficiency / Q000209:etiology
Lymphangiomyomatose. Fallbeschreibung und Literaturübersicht