Difference between revisions of "Pulmonary Langerhans cell histiocytosis"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Pulmonary Langerhans cell histiocytosis -- high mag.jpg | |||
| Width = | |||
| Caption = Langerhans cell histiocytosis of the lung. [[H&E stain]]. | |||
| Synonyms = eosinophilic granuloma (of the lung) | |||
| Micro = 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) | |||
| Subtypes = | |||
| LMDDx = | |||
| Stains = | |||
| IHC = Langerhans cells (CD1a +ve, S-100 +ve, CD207 +ve) | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Staging = | |||
| Site = [[lung]] - see ''[[medical lung diseases]]'' | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = [[smoking|smoker]], usually male 20-40 years old | |||
| Signs = +/-non-productive cough | |||
| Symptoms = +/-[[dyspnea]] | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = peribronchial nodules, upper lung zones or mid, multiple irregular cysts | |||
| Endoscopy = | |||
| Prognosis = good with smoking cessation | |||
| Other = | |||
| ClinDDx = non-pulmonary [[Langerhans cell histiocytosis]] | |||
| Tx = | |||
}} | |||
'''Pulmonary Langerhans cell histiocytosis''' is an uncommon [[smoking|smoking-related lung disease]]. | |||
It is also known as '''eosinophilic granuloma of the lung'''. | |||
The term ''Langerhans cell histiocytosis'' refers to several different diseases; a separate article deals with the other types of [[Langerhans cell histiocytosis]]. | |||
==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]]). | |||
Clinical - features:<ref name=pmid25473537>{{Cite journal | last1 = Martin | first1 = I. | last2 = Ballester | first2 = M. | last3 = Ruiz | first3 = Y. | last4 = Llatjós | first4 = R. | last5 = Alarza | first5 = F. | last6 = Molina | first6 = M. | title = Presentation of pulmonary Langerhans cell histiocytosis before the development of lung cysts. | journal = Respirol Case Rep | volume = 1 | issue = 2 | pages = 34-5 | month = Dec | year = 2013 | doi = 10.1002/rcr2.11 | PMID = 25473537 }}</ref> | |||
*Non-productive cough. | |||
*[[Dyspnea]]. | |||
*Typically males - 20-40 years old. | |||
*Smokers. | |||
===Subtypes=== | |||
Subtypes:<ref name=Ref_PPP234/> | |||
*Cellular form. | |||
*Fibrotic form. | |||
Note: | |||
*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). (???) | |||
DDx: | |||
*Non-pulmonary [[Langerhans cell histiocytosis]] - LCH is also found outside of the lung. | |||
===Images=== | |||
<gallery> | |||
Image: Pulmonary Langerhans cell histiocytosis -- low mag.jpg | PLCH - low mag. (WC) | |||
Image: Pulmonary Langerhans cell histiocytosis -- intermed mag.jpg | PLCH - intermed. mag. (WC) | |||
Image: Pulmonary Langerhans cell histiocytosis - alt -- intermed mag.jpg | PLCH - intermed. mag. (WC) | |||
Image: Pulmonary Langerhans cell histiocytosis -- high mag.jpg | PLCH - high mag. (WC) | |||
Image: Pulmonary Langerhans cell histiocytosis -- very high mag.jpg | PLCH - very high mag. (WC) | |||
</gallery> | |||
www: | |||
*[http://path.upmc.edu/cases/case299.html Pulmonary LCH & adenocarcinoma - several images (upmc.edu)]. | |||
==IHC== | |||
Langerhans cells: | |||
*S100 +ve.<ref name=Ref_PPP237>{{Ref PPP|237}}</ref> | |||
*CD1a +ve.<ref name=Ref_PPP237>{{Ref PPP|237}}</ref> | |||
*CD207 (AKA Langerin) +ve | |||
==See also== | |||
*[[Medical lung diseases]]. | |||
*[[Langerhans cell histiocytosis]]. | |||
*[[Smoking]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Medical lung diseases]] | |||
Latest revision as of 21:45, 15 April 2016
| 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 |
|
| |
| Clinical history | smoker, usually male 20-40 years old |
| Signs | +/-non-productive cough |
| Symptoms | +/-dyspnea |
| 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.
The term Langerhans cell histiocytosis refers to several different diseases; a separate article deals with the other types of Langerhans cell histiocytosis.
General
- Associated with smoking.[1]
- Not associated with systemic diseases of Langerhans cells (AKA Hand-Schueller-Christian disease).
Clinical - features:[2]
- Non-productive cough.
- Dyspnea.
- Typically males - 20-40 years old.
- Smokers.
Subtypes
Subtypes:[1]
- Cellular form.
- Fibrotic form.
Note:
- One form usually predominates.
Radiology
- Upper lung zones.
Microscopic
Features:[3]
- 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.
- ↑ Martin, I.; Ballester, M.; Ruiz, Y.; Llatjós, R.; Alarza, F.; Molina, M. (Dec 2013). "Presentation of pulmonary Langerhans cell histiocytosis before the development of lung cysts.". Respirol Case Rep 1 (2): 34-5. doi:10.1002/rcr2.11. PMID 25473537.
- ↑ 3.0 3.1 3.2 Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 237. ISBN 978-0443066313.