Clinical analysis of lymphangioleiomyomatosis / 中国医学科学院学报

ABSTRACT

<p><b>OBJECTIVE</b>To improve the diagnosis and treatment of lymphangioleiomyomatosis (LAM).</p><p><b>METHODS</b>The clinical data of four cases of lymphangioleiomyomatosis diagnosed in our hospital were analyzed and corresponding literature was reviewed.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSION</b>Clinicians should be familiar with the characteristics of LAM so that diagnosis and treatment can be made earlier to improve the prognosis.</p>