Pulmonary Langerhans cell histiocytosis

From Libre Pathology
Jump to: navigation, search
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.