肺淋巴管肌瘤病的临床及病理特点
卢韶华;谭云山;许建芳;宿杰·阿克苏;白春学;朱雄增
【期刊名称】《中华结核和呼吸杂志》 【年(卷),期】2009(032)009
【摘要】Objective To improve the understanding and diagnosis of pulmonary lymphangioleiomyomatosis (LAM). Methods Fifteen cases of LAM of our hospital were presented and 73 cases reported in domestic literature from 1993 to 2008 were reviewed. By means of histological and immunohistochemical(IHC)studies, the clinical and pathological features of LAM were analyzed. Results All the 88 cases were female, with an average age of onset at (37±9) years. The main clinical menifestations included dyspnea (83/88, 94%), hemoptysis (48/88, 54%), pneumothorax (41/88, 47%), and chylothorax (28/88, 32%). High resolution computerized tomography(HRCT) showed thin-walled air-filled cysts throughout both lungs. Pathological features showed cystic changes in the lung, and abnormal smooth muscle cells (LAM cells) lined the airways, bronchioles, lymphatics and blood vessels leading to airflow obstruction and replacement of the lung parenchyma by cysts. In the autopsy case, extrapulmonary organs (eg, kidney, lymph nodes and soft tissues) were also involved. Abnormal manifestations in abdomen,
including
renal
mass,
retroperitoneal
mass
and
retroperitoneal lymphadenopathy, were detected in 23 cases.
肺淋巴管肌瘤病的临床及病理特点
