Langerhans cell histiocytosis

Case contributed by Dr Vikas Shah

Presentation

Chronic shortness of breath.

Patient Data

Age: 30 years
Gender: Female
X-ray

Chest x-ray

The lung volumes are preserved. There is lateral peaking of the right hemidiaphragm, and adjacent to this there are staples in the right lower zone. There is a reticulonodular pattern of opacification but no airspace opacification.

The differential diagnosis is of a lung volume preserving chronic interstitial lung disorder: i.e. between Langerhans cell histiocytosis and lymphangiomyomatosis.

CT

CT

This CT indicates a predominant cystic pattern, with bizarre and odd shapes to the cysts. A few nodules are seen. Elsewhere on the CT, the bases were spared. She had previously had an open biopsy, this accounts for the staples and the minor hemidiaphragm peaking seen on the CXR.

Case Discussion

The diagnosis is Langerhans cell histiocytosis (LCH).

Key learning points:

  • early in the disease nodules predominate, later it is cysts
  • typical demographic: 20-40 year old females
  • sparing of extreme bases
  • lung volumes preserved
  • bizarre shape to cysts (round and regular in LAM)
PlayAdd to Share

Case information

rID: 57645
Published: 4th Jan 2018
Last edited: 14th Aug 2019
System: Chest
Inclusion in quiz mode: Included

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.