Yamamoto Kazumi, Anzai Fumio, Kusajima Kenji, Yamanishi Naoki, Yamada Masakazu
Department of Internal Medicine, Tachikawa Sougo General Hospital, 1-16-15 Nishikicho, Tachikawa, Tokyo 190-8578.
Intern Med. 2004 Aug;43(8):755-8. doi: 10.2169/internalmedicine.43.755.
A 29-year-old woman was admitted to our hospital with a 7-day history of elevated temperature to 39.5 degrees C associated with headache and nausea. She had been diagnosed with tuberous sclerosis complex 10 years earlier. Her unconsciousness progressed, and she was diagnosed as having aseptic meningoencephalitis. The next day, she had a generalized seizure with severe hemoptysis, and she suddenly fell into severe respiratory failure (PaO2/FiO2 = 76.9). Transbronchial lung biopsy revealed the findings of lymphangioleiomyomatosis. It was suggested that neurogenic pulmonary edema accompanied with venous flow obstruction by lymphangioleiomyomatosis lesions resulted in diffuse pulmonary hemorrhage with resultant gross hemoptysis accelerating to severe hypoxemia.
一名29岁女性因发热至39.5摄氏度达7天，伴有头痛和恶心而入住我院。她10年前被诊断为结节性硬化症复合体。她的意识障碍进展，被诊断为无菌性脑膜脑炎。第二天，她出现全身性癫痫发作并伴有严重咯血，随后突然陷入严重呼吸衰竭（动脉血氧分压/吸入氧分数值=76.9）。经支气管肺活检显示淋巴管平滑肌瘤病的表现。提示淋巴管平滑肌瘤病病变导致静脉血流受阻，继而引发神经源性肺水肿，导致弥漫性肺出血，进而出现大量咯血，加速发展为严重低氧血症。
