Case Report And Literature Review About Pulmonary Lymphangioleiomyomatosis

Objective: To summarize the experience of diagnosis and treatment of patients with pulmonary lymphangioleiomyomatosis and review relevant literatures,so as to enhance clinical understanding of the disease for clinicians.Methods: A retrospective analysis of data of one patients with pulmonary lymphangioleiomyomatosis(LAM)was made in 2012,in the Fifth Affiliated Hospital of Zunyi Medical College.The relevant literatures about LAM in recent years ware reviewed.The clinical features of LAM and experience of diagnosis and treatment were summarized.Results:(1)Basic data: female,the age of onset is 23 years old.(2)Clinical features:chest pain,chest pain,dyspnea and dyspnea appear in several cases.(3)Pulmonary manifestations: Chest X ray showed left pneumothorax,right pneumothorax,and left upper lung strip density shadow.The HRCT manifestations of the lung were right pneumothorax,a small amount of pleural effusion on the right side and diffuse cystic low-density areas in both lungs.(4)Extrapulmonary manifestations: HRCT in the middle and lower abdomen showed a retroperitoneal lymph node tumor.(5)Pulmonary function: severe restrictive ventilatory dysfunction and moderate pulmonary function decline.(6)Pathological findings: proliferation of spindle cells and epithelioid cells along the vasculature and small airway in the lung tissue;immunohistochemical: VEGF-D small(+),SMA(+ +),and HMB45(+).(7)Treatment: rapamycin treatment and symptomatic treatment.(8)Follow up: follow up for 62 months.Now the patient is working normally and has a child.Conclusion:(1)LAM is common in women of childbearing age.The main symptoms are progressive dyspnea,which can be accompanied by spontaneous pneumothorax,chylous pleural effusion and retroperitoneal lymph node swelling.(2)The HRCT and CT of the abdomen should be examined in time,when women of childbearing age have symptoms of chest tightness,chest pain,shortness of breath,and dyspnea.If the lung is diffuse cystic change,the possibility of this disease should be paid attention to,andthe pathology will be helpful for the diagnosis.(3)Sirolimus(rapamycin)has a good therapeutic effect on patients with pulmonary lymphangioleiomyomatosis.