Difference between revisions of "Pulmonary Langerhans cell histiocytosis"

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| LMDDx      =  
| LMDDx      =  
| Stains    =
| Stains    =
| IHC        = Langerhans cells (CD1a +ve, S-100 +ve, CD207 +ve
| IHC        = Langerhans cells (CD1a +ve, S-100 +ve, CD207 +ve)
| EM        =
| EM        =
| Molecular  =
| Molecular  =
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| Assdx      =
| Assdx      =
| Syndromes  =
| Syndromes  =
| Clinicalhx =
| Clinicalhx = [[smoking|smoker]], usually male 20-40 years old 
| Signs      =
| Signs      = +/-non-productive cough
| Symptoms  =
| Symptoms  = +/-[[dyspnea]]
| 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
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'''Pulmonary Langerhans cell histiocytosis''' is an uncommon [[smoking|smoking-related lung disease]].
'''Pulmonary Langerhans cell histiocytosis''' is an uncommon [[smoking|smoking-related lung disease]].


It is also known as '''eosinophilic granuloma of the lung'''.
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==
==General==
Line 39: Line 41:
*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 - 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/>
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.

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

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). (???)

DDx:

Images

www:

IHC

Langerhans cells:

  • S100 +ve.[3]
  • CD1a +ve.[3]
  • 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. 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. 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.