肺淋巴管肌瘤病临床诊治分析  

Clinical analysis of pulmonary lymphangioleiomyomatosis

作　　者：郑冠英[1,2] 何毅辉[3] 李瑞慧[1] 谢宝松[1,2] 林明[1,2] 陈愉生[1,2] 

机构地区：[1]福建省立医院呼吸内科,福州350001 [2]福建医科大学省立临床医学院,福州350001 [3]福建省立医院病理科,福州350001

出　　处：《创伤与急诊电子杂志》2016年第2期80-83,76,共5页Journal of Trauma and Emergency(Electronic Version)

摘　　要：目的 对肺淋巴管肌瘤病（pulmonary lymphangioleiomyomatosis,PLAM）的临床资料进行总结,提高对本病诊治的认识。方法 回顾性分析8例PLAM患者的临床资料,并随访5-92个月。结果 患者均为育龄期女性,临床症状以进行性呼吸困难最常见,误诊率100%。5例接受治疗的患者中：1例服用雷帕霉素2mg,每天1次,至今66个月,病情稳定;2例接受肺移植,术后生活质量提高,但分别于术后1年、3年死于肺部感染;2例接受安宫黄体酮治疗无效,死于呼吸衰竭。3例接受胸膜粘连固定术的气胸患者,术后术侧未再发气胸。结论 PLAM为罕见病,容易误诊,需进一步提高医师对该病的认识及警惕性。雷帕霉素可有效控制PLAM病情,对并发气胸患者,胸腔粘连固定术治疗可明显减少气胸复发。Objective To improve the understanding of pulmonary lymphangioleiomyomatosis （PLAM） by summarizing the clinical data of PLAM.Method Eight cases with PLAM diagnosed by lung biopsy or high resolution computerized tomography（HRCT） scanning were analyzed retrospectively and followed up from 5 to 92 months.Result The patients were all women of reproductive age. Progressive dyspnea was the major clinical manifestation of PLAM, followed by pneumothorax, chest tightness, cough, chylothorax, hemoptysis, chyloperitoneum and infection. The misdiagnosis rate was 100%. One patient taking rapamycin for 66 months was in stable condition; the quality of life improved in two patients after lung transplantation, while they died from pneumonia one and three years later respectively; two patients treated with medroxyprogesterone was invalid, and died from respiratory failure. Pleural adhesion fixation was performed in three cases with pneumothorax, and no postoperative recurrence was found on the same side.Conclusion PLAM is rare and easy to be misdiagnosed, which needs further understanding and vigilance. Rapamycin is considered to be effective for the disease. Pleural adhesion fixation is recommended for patients with pneumothorax.

关 键 词：肺淋巴管肌瘤病 临床特点 误诊 雷帕霉素 

分 类 号：R734.2[医药卫生—肿瘤]