Clinicopathologic analysis of pulmonary lymphangioleiomyomatosis.
- Author:
Yu LÜ
1
;
Song-Lin LIAO
;
Xiao-Nai TANG
;
Qiao YE
;
Lei ZHANG
;
Hong-Ying ZHAO
Author Information
- Publication Type:Journal Article
- MeSH: Actins; metabolism; Adult; Antigens, Neoplasm; Biopsy; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Lung; diagnostic imaging; pathology; Lung Neoplasms; diagnostic imaging; metabolism; pathology; Lymphangioleiomyomatosis; diagnostic imaging; metabolism; pathology; Matrix Metalloproteinase 2; metabolism; Melanoma-Specific Antigens; Middle Aged; Neoplasm Proteins; metabolism; Tomography, X-Ray Computed
- From: Chinese Journal of Pathology 2005;34(2):84-87
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVETo investigate the clinicopathological features of pulmonary lymphangioleiomyomatosis (PLAM).
METHODSBy means of HE and immunohistochemistry (SP method) studies, the clinical and pathological features of 5 PLAM cases were analyzed and the related literature reviewed.
RESULTSPLAM 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.
CONCLUSIONSPLAM 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.
