淋巴管平滑肌瘤病临床特点分析

Clinical Features of Lymphangioleiomyomatosis

目的总结淋巴管平滑肌瘤病(LAM)的临床特点,提高临床医生对该病的认识。方法通过具体分析作者本人经治的2例病例,并结合整理其他10例病例的临床表现及实验室检查结果,总结LAM的临床特点。结果 12例患者均为妇女,10例在育龄期内;10例有喘气、反复气胸、乳糜胸、咯血等呼吸道症状,并以此就诊;2例首诊症状为腹痛,其中1例伴有喘气,1例有乳糜胸病史。11例患者行肺功能检查,均为阻塞性通气功能障碍或以阻塞性通气功能障碍为主的混合性通气功能障碍。12例患者行CT检查,均有大小一致、分布均匀的薄壁囊腔样改变,2例伴有肺大泡,3例有纵隔淋巴结肿大,2例伴有盆腔肿块,1例有肾脏肿块,2例有腹膜后淋巴结肿大。所有患者确诊后给予抗雌激素治疗(安宫黄体酮),效果欠佳。结论 LAM是以育龄妇女发病,喘气、气胸、乳糜胸、咯血、腹痛及腹部盆腔包块为主要症状,伴有阻塞性通气功能障碍,薄壁囊腔样CT表现的肺疾病,治疗效果差。

Objective To discuss the clinical features of lymphangioleiomyomatosis（LAM）.Methods Clinical features of LAM were summarized through analyzing in details the clinical manifestations and laboratory results of 12 cases.Results All the 12 patients were female and 10 of them were in their reproductive period;Respiratory symptoms including recurrent pneumothorax,dysnpea,chylothorax,and hemoptysis were found in 10 cases as initial symptoms;initial symptoms for the other 2 cases were abdominal pain,yet one patient also complained associated dysnpea and the other patient had a history of chylothorax;11 patients underwent examination of lung functions,which showed either obstructive ventilatory disturbance or mixed ventilatory disturbance;all 12 patients underwent CT examinations and bilateral diffused cystic airspace changes were noted in all cases,pulmonary bullae were found in 2 cases,enlargement of mediastinal lymph nodes were found in 3 cases,pelvic masses were found in 2 cases,renal mass were found in 1 case,enlargement of retroperitoneal lymph nodes were found in 2 cases;all cases received anti-estrogen therapy which was barely effective.Conclusion LAM is a lung disease of women in reproductive period,the main symptoms of which include dysnpea,pneumothorax,chylothorax,hemoptysis,abdominal pain,and abdominal pelvic mass.Associated obstructive ventilatory disturbance and cystic airspace CT manifestations are also noted among LAM patients.The disease lacks effective treatment so far.