Lymphangioleiomyomatosis
From Health Encyclopedia
Contents |
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See for
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Broader Terms
lung disorder, lymphatic disorder
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Related Terms
orphan disease /drug, women's health
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Scope Note
rare lung disorder of unknown etiology mostly in women of childbearing age; proliferation of atypical smooth muscle cells in lungs, airways, blood and lymph vessels causing obstruction; symptoms include dyspnea and pneumothorax.
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Facts (generated by robot; please edit if you find it inaccurate)
- Lymphangioleiomyomatosis is abbreviated LAM.
- ukAIMS: Lymphangioleiomyomatosis is categorized by proliferation of abnormal smooth muscle cells (LAM cells) in the lungs and lymphatics and the presence of angiomyolipomas.
- Lymphangioleiomyomatosis is a rare condition characterized by a proliferation of the smooth muscle cells, mainly in the lymphatic system, giving rise to blockage of the thoracic duct with chylothorax and chyloascites.
- The exact cause of Lymphangioleiomyomatosis is not known.
- 5 Lymphangioleiomyomatosis is composed of a heterogeneous population of spindle cells with oval nuclei and large epithelioid cells; both cell types express smooth muscle actin and may express estrogen and progesterone receptors and HMB-45.
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