Desurmont S, Bauters C, Copin M C, Dewailly D, Tonnel A B, Wallaert B
Clinique des Maladies Respiratoires, Service de Pneumologie et Immuno-Allergologie, Hôpital A,-Calmette, CHRU, Lille.
Rev Mal Respir. 1996 Jul;13(3):300-4.
Pulmonary lymphangioleiomyomatosis is a rare and exclusively female disease which is oestrogen dependent and has a serious prognosis. We present two cases of young women aged 30 and 33 with the same clinical history of renal angiomyolipomas and recurring pneumothoraces. The computerised tomographic scans of the lung were strongly suggestive of LAM (there were fine wall cysts which were disseminated throughout both lung fields). The histological proof was provided by lung biopsy which was carried out during a thoracotomy for pleurectomies. The first patient who is currently asymptomatic and in excellent general health, was treated for three months with tamoxifen (Nolvadex), then for 20 months with 3.75 mg per month of triptoline (Decapeptyl), an agonist of GnRH. The second patient received triptoline in the same dose. After 40 months of treatment this patient was asymptomatic with satisfactory lung function tests. Agonists of GnRH (gonadotropic release hormone) seem to provide a useful alternative in the treatment of LAM.
肺淋巴管平滑肌瘤病是一种罕见的、仅见于女性的疾病，它依赖雌激素，预后严重。我们报告两例分别为30岁和33岁的年轻女性病例，她们有相同的肾血管平滑肌脂肪瘤和复发性气胸病史。肺部计算机断层扫描强烈提示为肺淋巴管平滑肌瘤病（两肺野均有弥漫分布的薄壁囊肿）。通过在开胸胸膜切除术中进行的肺活检提供了组织学证据。第一位患者目前无症状，总体健康状况良好，先用他莫昔芬（诺瓦得士）治疗三个月，然后用促性腺激素释放激素激动剂曲普瑞林（达必佳）每月3.75毫克治疗20个月。第二位患者接受相同剂量的曲普瑞林治疗。治疗40个月后，该患者无症状，肺功能测试结果令人满意。促性腺激素释放激素激动剂似乎为肺淋巴管平滑肌瘤病的治疗提供了一种有用的替代方法。
