Thursday, 14 October 2010

MRCP revision battle 29.6: Histiocytosis X

Histocytosis X, aka Langerhans-cell histiocytosis, is a group of disorders in which there is organ infiltration by granulomatous lesions containing clonally proliferated dendrintic (=Langerhams) cells.

The most commonly affected organs are lungs and bones.

Lung histiocytosis tends to affect young adults and is commonest in smokers.

The CXR shows multiple ring shadows with diffuse reticulo-nodular opacities affecting mainly the upper and mid zones.

Biopsy would show characteristic Birbeck granules.

Treatment is ?local excision, ?steroids.

Now onwards for a run of the last 3 short battles of the day, starting with Bartter's Syndrome...