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'''Pulmonary Langerhans cell histiocytosis''' is an uncommon [[smoking|smoking-related lung disease]]. | |||
It is also known as '''eosinophilic granuloma of the lung'''. | |||
==General== | |||
*Associated with smoking.<ref name=Ref_PPP234>{{Ref PPP|234}}</ref> | |||
*Not associated with systemic diseases of Langerhans cells ([[AKA]] [[Langerhans cell histiocytosis|Hand-Schueller-Christian disease]]). | |||
Subtypes:<ref name=Ref_PPP234/> | |||
*Cellular form. | |||
*Fibrotic form. | |||
One form usually predominates. | |||
===Radiology== | |||
*Upper lung zones. | |||
==Microscopic== | |||
Features:<ref name=Ref_PPP237>{{Ref PPP|237}}</ref> | |||
*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: | |||
*[http://path.upmc.edu/cases/case299.html Pulmonary LCH & adenocarcinoma - several images (upmc.edu)]. | |||
==IHC== | |||
*Langerhans cells: S100+ and CD1a+.<ref name=Ref_PPP237>{{Ref PPP|237}}</ref> | |||
==See also== | |||
*[[Medical lung diseases]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Medical lung diseases]] |
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