Art of Langerhans Cell Histiocytosis

Cell – Dendritic Cell

Langerhans Dendritic Macrophage
Ashley Davidoff MD
TheCommonVein.net

 

Langerhans Cell Histiocytosis
Langerhans Cell is a dendritic white cell with a wavy nucleus that creates granulomas and infiltrates the interstitium. It thus causes spiculated nodules that appear as spiculated nodules on CT
Ashley Davidoff
TheCommonVein.net
Smoking in Two Puff Harmony
From the series “People of Israel”
Ashley Davidoff MD
TheCommonVein.net
Membranous airways (respiratory bronchiole, alveolar ducts, alveolar sacs)
At the level of the membranous airways the effect is predominantly related to the loss of elasticity, and aberrant accumulation of smoking related macrophages.
The weakening and destruction results in emphysema and the abnormal accumulation of smoking related macrophages relates to DIP
Ashley Davidoff
TheCommonVein.net

The Bronchocentric Nodule

Image a shows a normal bronchiole.  Smoking excites the Langerhans cell which in turn  induces attracts  early cellular interstitial infiltrates of surrounding the bronchiole (b) including  lymphocytes, macrophages, eosinophils, plasma cells, and fibroblasts.  The cellular infiltrate progresses in a peribronchial pattern with mass effect on the bronchiole which becomes narrowed (c) and eventually disappears, a nodules of varying size manifest in the bronchiole pathway, sometimes round but often spiculated as the inflammatory reaction extends into the interstitium  (d) The wall of the bronchiole breaks down and the cellular infiltrate may undergo necrosis resulting in thick walled cavities, sometimes round in shape (e)  and sometimes with bizarre shapes (f) Eventually the inflammation recedes and a thin walled cyst remains (g ,h) 
Ashley Davidoff MD TheCommonVein.net

Florid Early Phase
Bronchocentric Nodules = Inflammatory Changes Around the Bronchus 

Smoking excites the Langerhans cell which in turn induces and attracts early cellular interstitial infiltrates which surround the bronchiole. The diagram shows a bronchiole surrounded by an acute cellular inflammatory response
Ashley Davidoff MD TheCommonVein.net lungs-0722

Tree in Bud and Peribronchial Nodules with a Hint of Central Cavitation ie Bronchocentricity

As the inflammatory response progresses it has mass effect on the bronchiole causing the bronchiole to become narrowed.
Ashley Davidoff MD TheCommonVein.net lungs-0723
The inflammatory response is often aggressive and may infiltrate the surrounding interstitium resulting in a spiculated appearance. As the inflammatory response progresses it has mass effect on the bronchiole causing the bronchiole to become narrowed.
Ashley Davidoff MD TheCommonVein.net lungs-0723b

 

Stellate Appearance Because of Infiltration of the Inflammatory Process into the Interstitium

It may become a Solid Nodule as It Obliterates the Lumen

As the inflammatory response progresses it has mass effect on the and may eventually occlude the bronchiole becoming a nodule.
Ashley Davidoff MD TheCommonVein.net lungs-0724b
The inflammatory response is often aggressive and may infiltrate the surrounding interstitium resulting in a spiculated appearance
Ashley Davidoff MD TheCommonVein.net lungs-0724

 

Wall Starting to Break Down Creating a  Thick Walled Cyst 

The wall of the bronchiole breaks down and the cellular infiltrate may undergo necrosis resulting in thick -walled cavities, sometimes round in shape.
Ashley Davidoff MD TheCommonVein.net lungs-0725

Sometimes with Bizarre Shapes

The wall of the bronchiole breaks down and the cellular infiltrate may undergo necrosis resulting in thick -walled cavities, sometimes bizarre in shape.
Ashley Davidoff MD TheCommonVein.net lungs-0726

 

With Time Inflammation Settles and There is Thinning of the Walls of the Cysts

As the inflammatory response subsides, thin-walled cavities evolve.
Ashley Davidoff MD TheCommonVein.net lungs-0728b -hi res
As the inflammatory response subsides, thin-walled cavities evolve, sometimes bizarre in shape.
Ashley Davidoff MD TheCommonVein.net lungs-0728b