lymphangiomyomatosis

lymphangiomyomatosis

 [lim-fan″je-o-mi″o-mah-to´sis]
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.

lym·phan·gi·o·lei·o·my·o·ma·to·sis

(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.

lymphangiomyomatosis

/lym·phan·gio·my·o·ma·to·sis/ (lim-fan″je-o-mi″o-mah-to´sis) 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.
References in periodicals archive ?
Pulmonary lymphangiomyomatosis (LAM) associated with both renal and hepatic angiomyolipomas (AMLs) in a TSC patient is very rare.
Perivascular epithelioid cell tumors (PEComas) of the kidney are a group of tumor family covering classical angiomyolipoma (AML), microscopic AML (microhamartoma), intraglomerular lesions, cystic AML, epithelioid AML, oncocytoma-like AML and lymphangiomyomatosis of the renal sinus.
3) As noted by Davis et al, (2) a dominant feature in all cases of AMLEC is a prominent lymphatic channel network in the smooth muscle component forming slitlike branched and curvilinear spaces strongly resembling those seen in lymphangiomyomatosis.
Major Criteria Minor Criteria Facial angiofibromas or Multiple randomly distributed forehead plaque pits in dental enamel Ungual or periungual fibroma Hamartomatous rectal polyps (Non-traumatic) Bone cysts Hypomelanotic macules (>3) Cerebral white matter Shagreen patch (connective migration tracts tissue naevus) Gingival fibromas Cortical tuber Nonrenalhamartoma Subependymal nodule Retinal achromic patch Subependymal giant cell astrocytoma Confetti skin lesions Multiple retinal nodular hamartomas Multiple renal cysts Cardiac rhabdomyoma, single or multiple Nonrenal hamartomas Pulomary lymphangiomyomatosis Renal angiomyolipoma.
Major features of TSC include cutaneous lesions such as adenoma sebaceum, more than three hypomelanotic macules, shagreen patch, and periungual fibromas; cortical tubers, subependymal nodules, retinal hamartomas, heart rhabdomyomas, renal angiomyolipomas, and lung lymphangiomyomatosis.
Lymphangiomyomatosis typically presents in the third or fourth decade of life.
The four main causes of chylothorax include: malignancy; trauma; idiopathic; and miscellaneous causes such as thrombosis of the superior vena cava or subclavian vein, cirrhosis and rarely, pulmonary lymphangiomyomatosis.
He had a history of right ureterolithotomy and diabetes, although no history of tuberous sclerosis, lymphangiomyomatosis, renal cancer or renal cyst was present.
In comparison, lymphangiomyomatosis (LAM) shows larger thin-walled cysts that are diffusely distributed in the lungs and eventually involve the entire lung parenchyma.
Mrs Priest, 61, of Colders Drive, suffers from a respiratory disease, pulmonary lymphangiomyomatosis.
INTRODUCTION: Lymphangiomyomatosis is a rare, diffuse and progressive lung disease that affects young women of child bearing age.