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Difference between revisions of "Pulmonary Langerhans cell histiocytosis"
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Pulmonary Langerhans cell histiocytosis (view source)
Revision as of 21:45, 15 April 2016
, 21:45, 15 April 2016no edit summary
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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 = | ||
| Line 19: | Line 19: | ||
| 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 | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = non-pulmonary [[Langerhans cell histiocytosis]] | ||
| Tx = | | Tx = | ||
}} | }} | ||
'''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== | ||
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*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. | ||
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**+/-Eosinophilia (may be rare) - '''significantly narrow DDx'''. | **+/-Eosinophilia (may be rare) - '''significantly narrow DDx'''. | ||
**Chronic inflammatory cells (lymphocytes). (???) | **Chronic inflammatory cells (lymphocytes). (???) | ||
DDx: | |||
*Non-pulmonary [[Langerhans cell histiocytosis]] - LCH is also found outside of the lung. | |||
===Images=== | ===Images=== | ||