OBJECTIVETo improve the diagnosis and treatment of lymphangioleiomyomatosis (LAM).

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

RESULTSLymphangioleiomyomatosis, 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.

CONCLUSIONClinicians should be familiar with the characteristics of LAM so that diagnosis and treatment can be made earlier to improve the prognosis.