肺淋巴管平滑肌瘤病的诊断和治疗

Diagnosis and therapy of pulmonary lymphangioleiomyomatosis

目的 探讨肺淋巴管平滑肌瘤病（PLAM）的临床表现、影像学及病理学特点、诊断和治疗方法等,以提高对本病的诊疗效果.方法 回顾性分析上海交通大学附属第一人民医院胸外科近年收治的5例PLAM患者的临床资料,并复习文献.结果 5例患者均为女性,年龄22～49岁,平均36.6岁.患者主要表现为劳力性呼吸困难、咯血、反复发作的气胸及乳糜胸,1例患者合并肾肌脂瘤.胸部高分辨率CT （HRCT）检查显示：双肺弥漫分布薄壁含气囊腔.肺功能检查主要为阻塞性或混合性通气功能障碍.有4例患者经病理确诊.1例终末期患者行左肺移植术,术后肺功能明显改善,4年后因自体肺过度膨胀再行右肺切除术.1例终末期患者给予抗雌激素等保守治疗,病情缓解.1例终末期患者放弃进一步治疗,5个月后死于呼吸衰竭.1例患者主要表现为气胸反复发作,行肺大疱切除术后已随访2年,气胸未再复发.1例患者表现为气胸首次发作,行肺大疱切除,术后恢复良好.结论 PLAM是一种罕见肺部囊性病变,胸部HRCT具有特征性表现,病理为诊断的金标准.该病目前缺乏有效的内科治疗手段,肺移植是终末期患者最有效的治疗方法.

Objective To discuss the clinical manifestations,radiological and pathological features,diagnostic and therapeutic methods of pulmonary lymphangioleiomyomatosis （PLAM）,and to improve diagnostic and therapeutic levels of PLAM.Methods Clinical data of 5 cases with PLAM was analyzed retrospectively and the relevant literature was reviewed.Results Five patients suffered from PLAM were the women in their reproductive aged,from 22 years to 49 years （mean 36.3 years）.The major manifestation include exertional dyspnea,hemoptysis,recurrent spontaneous pneumothorax and ehylothorax.One case was found to be complicated with renal angiomyolipoma.High-resolution computerized tomography （H-RCT） findings of PLAM were certain characteristics：bilateral diffuse thin walled cystic airspaces change.Pulmonary function test showed obstructive or compound ventilative defect and hypoxemia.Four patients were pathologically confirmed.An end-stage patient underwent a single left lung transplantation.After transplantation,the lung function significantly improved.She underwent right pneumonectomy 4 years later for contralateral lung over-expansion.One end-stage patient were treated with anti-estrogen and other conservative treatment,in remission.One end-stage patient gave up further treatment,five months later died of respiratory failure.One case whose major manifestation was recurrent spontaneous pneumothorax underwent pulmonary bullectomy,2years-follow-up result was satisfactory.One case showed pneumothorax first attack underwent pulmonary bullectomy,postoperative recovery.Conclusion PLAM is a rare lung cystic lesions,chest HRCT findings are certain characteristics,and pathology is the gold standard for diagnosis.There is no effective medical therapy for PLAM at present,lung transplantation is the most effective way to treat the end-stage patients.