Lymphangioleiomyomatosis

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Contents

See for

lymphangiomyomatosis

Broader Terms

lung disorder, lymphatic disorder

Related Terms

orphan disease /drug, women's health

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.

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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