Difference between revisions of "Pulmonary Langerhans cell histiocytosis"
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| Line 24: | Line 24: | ||
| Prevalence = uncommon | | Prevalence = uncommon | ||
| Bloodwork = | | Bloodwork = | ||
| Rads = upper lung zones | | Rads = peribronchial nodules, upper lung zones or mid, multiple irregular cysts | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = good with smoking cessation | | Prognosis = good with smoking cessation | ||
| Line 39: | Line 39: | ||
*Not associated with systemic diseases of Langerhans cells ([[AKA]] [[Langerhans cell histiocytosis|Hand-Schueller-Christian disease]]). | *Not associated with systemic diseases of Langerhans cells ([[AKA]] [[Langerhans cell histiocytosis|Hand-Schueller-Christian disease]]). | ||
Clinical: | |||
*Non-productive cough. | |||
*[[Dyspnea]]. | |||
===Subtypes=== | |||
Subtypes:<ref name=Ref_PPP234/> | Subtypes:<ref name=Ref_PPP234/> | ||
*Cellular form. | *Cellular form. | ||
*Fibrotic form. | *Fibrotic form. | ||
One form usually predominates. | Note: | ||
*One form usually predominates. | |||
==Radiology== | ==Radiology== | ||
*Upper lung zones. | *Upper lung zones. | ||
Revision as of 06:34, 23 December 2015
| 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 | peribronchial nodules, upper lung zones or mid, multiple irregular cysts |
| Prognosis | good with smoking cessation |
| Clin. DDx | non-pulmonary Langerhans cell histiocytosis |
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).
Clinical:
- Non-productive cough.
- Dyspnea.
Subtypes
Subtypes:[1]
- Cellular form.
- Fibrotic form.
Note:
- 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:
DDx:
- Non-pulmonary Langerhans cell histiocytosis - LCH is also found outside of the lung.
Images
PLCH - low mag. (WC)
PLCH - intermed. mag. (WC)
PLCH - intermed. mag. (WC)
PLCH - high mag. (WC)
PLCH - very high mag. (WC)
www:
IHC
Langerhans cells:
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 2.2 Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 237. ISBN 978-0443066313.