Pulmonary Langerhans cell histiocytosis
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Pulmonary Langerhans cell histiocytosis is an uncommon smoking-related lung disease.
It is also known as eosinophilic granuloma of the lung.
General
- Associated with smoking.[1]
- Not associated with systemic diseases of Langerhans cells (AKA Hand-Schueller-Christian disease).
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). (???)
- Langerhans cells - key feature:
Images:
IHC
- Langerhans cells: S100+ and CD1a+.[2]
See also
References
- ↑ 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.
- ↑ 2.0 2.1 Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 237. ISBN 978-0443066313.