Surgical Pathology Criteria

Pulmonary Langerhans Cell Histiocytosis

Differential Diagnosis

Langerhans Cell Histiocytosis Usual Interstitial Pneumonia
Scars frequently stellate and centrilobular with extension to subpleura Irregular subpleural scarring
Honeycomb change only occasionally seen Honeycomb change frequent
Frequently some typical active LCH lesions present Lacks peribronchiolar nodules of Langerhans cells
Langerhans cells are positive for CD1a and langerin CD1a and langerin negative
HRCT shows centrilobular nodule and cyst pattern HRCT shows honeycombing, peripheral accentuation

 

Langerhans Cell Histiocytosis Hypersensitivity Pneumonitis
Lacks granulomas and giant cells Granulomas and giant cells generally present but may be infrequent in late stage
Smoking history only Possible exposure history
CD1a and langerin positive Langerhans cells present No Langerhans cells
Late fibrotic stage may be impossible to separate if active foci cannot be found

 

Respiratory Bronchiolitis Associated Interstitial Lung Disease Langerhans Cell Histiocytosis
Predominantly intra-alveolar process Predominantly interstitial process, intra-alveolar macrophages only focally present
Eosinophils rare Eosinophils may be numerous
No cystic change Cystic change frequent
Lacks scarring Produces stellate scars
Histiocytes with round nuclei Histiocytes with folded nuclei
Macrophages CD1a and langerin negative Langerhans cells positive for CD1a and langerin
Both are highly associated with smoking, thus foci or respiratory bronchiolitis are expected to be present in the lung tissue of cases of Langerhans cell histiocytosis

 

Langerhans Cell Histiocytosis Desquamative Interstitial Pneumonia
Intra-alveolar macrophages only focally present Diffuse intra-alveolar macrophages
Temporal heterogeneity Temporally uniform
Focal or multifocal disease Diffuse disease
Numerous eosinophils Occasional eosinophils
Cystic change frequent No cystic change
Produces stellate scars Lacks scarring
Histiocytes with folded nuclei Histiocytes with round nuclei
Langerhans cells positive for CD1a and langerin Macrophages CD1a and langerin negative

 

Langerhans Cell Histiocytosis Chronic Eosinophilic Pneumonia
Predominantly interstitial process, intra-alveolar macrophages only focally present Predominantly intra-alveolar process
Composed of Langerhans cells with variable numbers of eosinophils Prominent eosinophils, no Langerhans cells
Produces stellate scars Lacks scarring
Histiocytes with folded nuclei Histiocytes with round nuclei
S100, CD1a, langerin positive S100, CD1a, langerin negative

 

Langerhans Cell Histiocytosis Eosinophilic Pleuritis
Predominantly a parenchymal interstitial process Pleural process, no parenchymal involvement
Langerhans cells with variable numbers of eosinophils Predominantly eosinophils with proliferating mesothelial cells
HRCT shows centrilobular nodule and cyst pattern HRCT shows localized pleural lesion

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