Pulmonary Langerhans cell histiocytosis

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Pulmonary Langerhans cell histiocytosis is an uncommon smoking-related lung disease.

Pulmonary Langerhans cell histiocytosis
Diagnosis in short

Langerhans cell histiocytosis of the lung. H&E stain.

Synonyms eosinophilic granuloma (of the lung)

LM cellular peribronchiolar nodules with Langerhans cells (pale staining nucleus (H&E) with nuclear infolding - "crumpled tissue paper" appearance), +/-smoker's macrophages (brown pigmented airspace macrophages), +/-eosinophilia (typical - may be rare)
IHC Langerhans cells (CD1a +ve, S-100 +ve, CD207 +ve
Site lung - see medical lung diseases

Prevalence uncommon
Radiology upper lung zones
Prognosis good with smoking cessation

It is also known as eosinophilic granuloma of the lung.

General

Subtypes:[1]

  • Cellular form.
  • Fibrotic form.

One form usually predominates.

Radiology

  • Upper lung zones.

Microscopic

Features:[2]

  • Cellular peribronchiolar nodules with:
    • Langerhans cells - key feature:
      • Pale staining nucleus (H&E) with nuclear infolding - "crumpled tissue paper" appearance.
    • +/-Smoker's macrophages (brown pigmented airspace macrophages).
    • +/-Eosinophilia (may be rare) - significantly narrow DDx.
    • Chronic inflammatory cells (lymphocytes). (???)

Images:

IHC

Langerhans cells:

  • S100 +ve.[2]
  • CD1a +ve.[2]
  • CD207 (AKA Langerin) +ve

See also

References

  1. 1.0 1.1 Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 234. ISBN 978-0443066313.
  2. 2.0 2.1 2.2 Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 237. ISBN 978-0443066313.