Poh S C, Wang Y T
Department of Medicine III, Tan Tock Seng Hospital, Singapore.
Singapore Med J. 1991 Aug;32(4):258-61.
Lymphangioleiomyomatosis is a rare devastating disease affecting women mostly of child-bearing age. It presents with spontaneous pneumothorax, chylous effusions, hemoptysis and progressive breathlessness. Most patients die from respiratory failure within 10 years. There are no controlled studies on the efficacy of various treatment regimens. We report our experience with progesterone therapy in three patients. Two failed to respond, one died about 11 years after presentation and another after 5 1/2 years. The third patient has survived 11 years after onset of disease.
淋巴管平滑肌瘤病是一种罕见的、严重影响育龄女性的疾病。其症状包括自发性气胸、乳糜性胸腔积液、咯血和进行性呼吸困难。大多数患者在10年内死于呼吸衰竭。目前尚无关于各种治疗方案疗效的对照研究。我们报告了3例患者接受孕激素治疗的经验。其中2例无效，1例在出现症状后约11年死亡，另1例在5年半后死亡。第3例患者自疾病发作后已存活11年。
