[Clinicopathologic analysis of pulmonary lymphangioleiomyomatosis].
OBJECTIVE
To investigate the clinicopathological features of pulmonary lymphangioleiomyomatosis (PLAM).


METHODS
By means of HE and immunohistochemistry (SP method) studies, the clinical and pathological features of 5 PLAM cases were analyzed and the related literature reviewed.


RESULTS
PLAM was a rare lung disease of unknown etiology and was restricted to females who were generally pre-menopausal. Pathological features showed abnormal smooth muscle cells (LAM cells) line the airways, lymphatics and blood vesssels leading to airflow obstruction and replacement of the lung parenchyma by cysts. LAM cells were positive for HMB45. Clinically the disease was categorized by dyspnoea, haemoptysis, recurrent pneumothoraces and chylous effusions.


CONCLUSIONS
PLAM should be considered when recurrent pneumothorax, haemoptysis and dyspnoea occur in females. Pathologic examination of lung tissue biopsy is required for confirmation of PLAM diagnosis.
Lü
Yu
Y
Department of Pathology, Beijing Chaoyang Hospital, Capital University of Medical Sciences, Beijing 100020, China.
Liao
Song-Lin
SL
Tang
Xiao-Nai
XN
Ye
Qiao
Q
Zhang
Lei
L
Zhao
Hong-Ying
HY
D000199:Actins / Q000378:metabolism; D000328:Adult; D000951:Antigens, Neoplasm; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology*; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D018192:Lymphangioleiomyomatosis / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D020778:Matrix Metalloproteinase 2 / Q000378:metabolism; D058950:Melanoma-Specific Antigens; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000378:metabolism; D014057:Tomography, X-Ray Computed
D000199:Actins; D000951:Antigens, Neoplasm; D020778:Matrix Metalloproteinase 2; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins; C498862:Acta2 protein, rat