[Lymphangioleiomyomatosis].
Lymphangioleiomyomatosis (LAM) is a rare disease, which only affects women of childbearing age. A case of LAM in a 36-year-old woman is presented. Patho-anatomical findings are smooth muscle hyperplasia leading to obstruction of lymphatics (possibly resulting in chylothorax), blood vessels (possibly resulting in haemoptysis) and bronchi (possibly resulting in emphysema and pneumothorax). High resolution computed tomography (HRCT) of the chest can show the typical multiple small cysts and bullous emphysema distributed diffusely throughout both lungs together with interstitial fibrosis. Treatment of the disease aims at an inhibition of the presumed growth-promoting effect of oestrogen on the smooth muscle cells. However, progression is often seen in spite of treatment.
Medicinsk-lungemedicinsk afdeling Y, Amtssygehuset i Gentofte.
P S
PS
Clementsen
K
K
Folke
P
P
Faurschou
D000328:Adult; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology; D018192:Lymphangioleiomyomatosis / Q000000981:diagnostic imaging* / Q000473:pathology; D014057:Tomography, X-Ray Computed
Lymphangioleiomyomatosis.