(redirected from Pulmonary lymphangioleiomyomatosis)


a progressive disorder of women of child-bearing age, marked by nodular and diffuse interstitial proliferation of smooth muscle in the lungs, lymph nodes, and thoracic duct. Called also lymphangioleiomyomatosis.


(lim-fam'jē-ō-lī'ō-nū'ō-mă-tō'sis), [MIM*606690]
A rare disorder of unknown etiology seen in women of reproductive age and in patients of either sex with tuberous sclerosis. Pulmonary complications are due to hamartomatous proliferation of smooth muscle cells preferentially along bronchovascular structures resulting in obliteration of the airways and consecutive development of cysts in the lungs. Usually progressive, leading to death from respiratory failure. Treatment by lung transplantation has been successful.


/lym·phan·gio·leio·myo·ma·to·sis/ (lim-fan″je-o-li″o-mi″o-mah-to´sis) lymphangiomyomatosis.
References in periodicals archive ?
The association of pulmonary lymphangioleiomyomatosis with renal and hepatic angiomyolipomas in a prepubertal girl: A previously unreported entity.
High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex.
Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease affecting fertile women and is characterized by progressive dyspnea on exertion.
Pulmonary lymphangioleiomyomatosis (LAM) is an uncommon lung disease primarily affecting women of childbearing age.
Cathepsin-k expression in pulmonary lymphangioleiomyomatosis.
Combination of pulmonary lymphangioleiomyomatosis with skin leiomyomatosis and uterine leiomyoma] Arkh Patol 2001;63:39-44.
Treatment of pulmonary lymphangioleiomyomatosis using a GnRH agonist.
Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous oestrogen used for infertility treatment.
Pulmonary lymphangioleiomyomatosis and steroid receptors: an immunocytochemical study.
Mutation analysis of the TSC1 and 7TC2 genes in Japanese patients with pulmonary lymphangioleiomyomatosis.
Pulmonary lymphangioleiomyomatosis, a disease of women, is characterized by:
Pulmonary lymphangioleiomyomatosis (LAM), a disease of women, is characterized by (1) proliferation of abnormal smooth muscle cells (LAM cells) in the pulmonary interstitium and along axial lymphatics and lymph nodes of the thorax and abdomen, (2) parenchymal cysts throughout the lungs, and (3) a high incidence of angiomyolipomas.

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