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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 ?
Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis.
Lymphangioleiomyomatosis is a slowly progressive disease that will eventually lead to respiratory failure and even death.
A chest CT showed lymphangioleiomyomatosis in both lungs and an abdominal CT on the second postoperative revealed bilateral renal angiomyolipomas.
Lymphangioleiomyomatosis (LAM) is a rare progressive lung disease of unknown etiology that occurs exclusively in women during their reproductive and menopause years.
A snapshot of the global therapeutic scenario for Lymphangioleiomyomatosis.
Small Molecule to Inhibit Src Tyrosine Kinase for Lymphangioleiomyomatosis 202
2-4) Some of those diseases are well described and have clearly recognizable pathologic features, such as lymphangioleiomyomatosis or malignancy.
2 Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis
Lymphangioleiomyomatosis (LAM) is a disease that predominantly affects young females and generally progresses to respiratory failure and death.
42) Recurrence of the primary disease has been described with many conditions, including sarcoidosis, lymphangioleiomyomatosis, Langerhans cell histiocytosis, talc granulomatosis, diffuse panbronchiolitis, and alveolar proteinosis.