2 Cases Report Of Pulmonary Lymphangioleiomyomatosis And Literatures Review

Objective: To investigate the clinical characteristics of pulmonary lymphangioleiomyomatosis(LAM) and summarize the experience of diagnosis and treatment.Methods: 2 cases of LAM admitted in the first affiliated hospital of guangxi medical university in 2010 and 122 cases reported in domestic literature from 1993 to 2010 are studied from clinical manifestations, radiological and pathological features, pulmonary functional and treatment.Results:(1) General information:①2 patients are both female, age of onset is 13 and 40 years old respectively.②Among the 122 cases of LAM, 98% are female, and 2% are male, with an average age of onset at 5-65(35±8)years.(2) Clinical manifestations:①The mainly manifestation of 2 patients is progressive dyspnea on exertion. In addition, one patient shows pleural effusion and pericar- dial effusion. The other shows recurrent pneumothorax.②Among the 122 cases of LAM,the main clinical manifestations included dyspnea(93%),cough (50%), pneumothorax(41%),chylothorax(41%), hemoptysis(34%). At first, most people went to hospital because dyspnea(69%). (3) extrapulmonary manifestation:① 2 patients of our hospital have not extrapulmonary manifastations.②Among the 122 cases, the most commonest extrapulmonary manifestation is renal angiomy- olipomas in 34 cases who have abdominal examination(44%). (4) Laboratory examination:①Pleural effusion and pericardial effusion of a patient in our hospital are confirmed as chylous by biochemical examination.②Among the 122 cases, pleural effusion of 50 cases are confirmed as chylous by biochemical examination. At the same time, 7 cases of them exist chylous abdominal effusion, and 1 case exist chylous pericardial effusion. (5) High resolution computerd tomograghy(HRCT)of lung:①One case shows specific multiple thin-walled well-defined cysts scattered in the lungs and pneumothorax on right. The other showes unspecific interstitial changes, reticule-infiltrate, pleural effusion on left, and pericardial effusion.②Among the 122 cases, 90% HRCT showed thin-walled cysts throughout both lungs, and 10% showed nonspecific interstitial changes. (6)pulmonary functional test:①The pulmonary function test of 2 cases are both mixed ventilation dysfunction②Among the 122 cases, the obstructive ventilation dysfunction and diffusion impairment was observed in 57% patients respectively. Mixed ventilation dysfunction was observed in 33% patients. (7)Pathology:①Smooth muscle-like cells(LAM cells)are found to line the airways, bronchioles, lymphatics and blood vessel in 2 cases. At the same time, one also shows multiple hyperplasia of alveolar epithelial; 2 patients underwent immunohistochemical staining, both of them are positive in SMA,and 1 positive in HMB-45, the other is negative.②Among the 122 cases, 89% pathological features showed smooth muscle-like cell proliferation around airway and vessel, cystic and hyperinflationary changes in the lung. Some patients underwent immunohistochemical staining, part of them are positive in SMA, HMB-45 and Actin.(8)misdiagnosis:①2 patients are all misdiagnosed,the common misdiagnosis is pneumothorax or tuberculosis. The time from onset of symptoms to diagnosis are both 3 years.②Among the 122 cases, the common misdiagnosis is bronchiectasis, chronic bronchitis, interstitial disease, pulmonary tuberculosis and so on. The average misdiagnosis time was 31months(1-252 months). (9) Antiestrogen therapy effect is poor and rapamycin can be effective to LAM.Conclusions:(1) LAM is primarily occurring in women at reproductive age, and characterized by progressive dyspnoea and recurrent pneumothorax, chylous collections and angiomyolipoma in the kidneys. (2)It is often be misdiagnosed. To women at reproductive age,whose clinical manifestations as above and HRCT of lung show multiple thin-walled cysts scattered in the lungs, lung biopsies is necessary to diagnosis.(3)Antiestrogen therapy need to go on to research. Rapamycin can keep the pulmonary function stable, but if stop taking medicine, the effectiveness can not be maintained. Lung transplantation has been established as persistently effective therapy for end-stage pulmonary LAM.