[Clinical analysis of lymphangioleiomyomatosis].
OBJECTIVE
To improve the diagnosis and treatment of lymphangioleiomyomatosis (LAM).


METHODS
The clinical data of four cases of lymphangioleiomyomatosis diagnosed in our hospital were analyzed and corresponding literature was reviewed.


RESULTS
Lymphangioleiomyomatosis, a rare disorder of unknown cause that occured almost exclusively in women of child bearing ages, was characterized microscopically by atypical smooth muscle proliferation. It could occur as a sporadic diseases or as a part of tuberous sclerosis complex (TSC). LAM was associated with progressive dyspnea, recurrent spontaneous pneumothoraces, chylous effusions, and hamartomas.


CONCLUSIONS
Clinicians should be familiar with the characteristics of LAM so that diagnosis and treatment can be made earlier to improve the prognosis.
Gao
Peng
P
Department of Respiratory Disease, PUMC Hospital, CAMS and PUMC, Beijing 100730, China.
Huang
Rong
R
Cai
Bai-qiang
BQ
Liu
Hong-rui
HR
D000293:Adolescent; D000328:Adult; D001707:Biopsy, Needle; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed