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Pulmonary Langerhans Cell Histiocytosis (PLCH)

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Alright, let's talk about longer han cell histiocytosis.

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And so this looks so different from the other types

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of diffuse cystic lung disease.

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These are ugly cyst. They are.

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So look at this, look at these ugly cysts.

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So sometimes they're thin walled,

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sometimes they're thick walled like this

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and like that they start to coalesce into each other

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and so they cause these bizarre shaped cysts.

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So if you're taking a test for those of you guys

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who are still taking tests for radiology

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or medicine, that word, that buzzword is bizarre shaped.

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And these are indeed bizarre shape.

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'cause what they do is these cysts start,

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they run into each other and they cause these weird shapes

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like that like little animals or continents.

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They can be round in shape

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but sometimes they can be oval as well.

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As we seen here, these cysts actually emanate from

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central lobular nodules.

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So in my talk on diffuse nodule lung disease,

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I talk about the differential of diagnosis

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for central lobular nodules.

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And one of the things on their lower down is long hind

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cell histiocytosis.

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And so early in disease they pre, they manifest

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as these central lobular nodules

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and over time they start to cavitate out

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and then cause a diffuse cystic lung disease.

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So longhand cell histiocytosis is both on the differential

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diagnosis for diffuse cystic lung disease

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but also diffuse nodule lung disease.

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And so when they start the caviate, we call these Cheerios.

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So people call these Cheerios

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nodules and that's what they look like.

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Little cheerios in the lungs,

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tiny little cavities like that.

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And so LCH loves to do that.

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LCH also is an upper lung proponent disease.

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It could certainly extend down to the lung bases,

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but almost always the upper lobes would be more severely

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affected than the lower lobes.

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And classically, I'd say at least 90, 95%

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of the time we'll spare the CAA Phrenic socy.

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As we see here. Another patient

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with lung hand cell histiocytosis smic socy are spared.

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Another example here of these weird bizarre shaped cysts,

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ugly cysts, some thick wall, some thin wall.

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And we see concomitant central lo nodules

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and some Cheerios nodules as well.

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More examples of longer han cell histiocytosis presenting

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with these bizarre shaped cysts.

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Beautiful examples here. You could almost feel like these

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cysts, you could imagine

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where the cyst actually emanated from

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and how they're now running into each other.

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Almost like puddles.

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Again, this is an upper lung preponderant

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disease as we see here.

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Remember this is a smoking related lung disease in adults.

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There is also longer on cell histic cytosis

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that we see mainly in pediatric patients.

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Uh, that's a a little bit

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of a different beast in setting of adults.

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This is a smoking related lung disease.

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Some people actually say that this,

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this is almost like a malignant disease.

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So you're getting these cells

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which are growing outta control.

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And so that's another way to think of it.

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But from the standpoint of a diagnostic general radiologist,

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I think we can just remember this is a

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smoke related lung disease.

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We do. Sometimes we will get these areas

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of a complex pattern again within the upper aspect of lungs

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Where it almost looks like emphysema plus cyst,

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plus reticulation plus nodules.

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You guys see this, the nodules de marketed

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by the orange arrows.

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And so if the patient has history of significant smoking,

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this is a home run for longhand cell his of cytosis.

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So when LCH starts to quote unquote burn out,

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so the inflammation starts to go away, a subset

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of these cases can actually develop pulmonary fibrosis.

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So when you get the pulmonary fibrosis superimposed on the

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underlying diffuse cystic lung disease, oftentimes

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with associated emphysema 'cause they're also smoking

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and smoking related empa is quite common.

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And then you have these, these underlying little nodules

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or cavitary cheerios like nodules,

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it will manifest like this.

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It just looks like it looks very

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complex, kind of hurts your brain.

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Just looks like a lot of reticular abnormality

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here with cyst.

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If the patient has history of smoking

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and if there's relative basal sparing, put LCH very,

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very high on your differential diagnosis.

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I think most of the time we don't even biopsy these patients

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anymore 'cause the imaging pattern is

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so darn characteristic.

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Beautiful schizophrenic angle sparing classic.

Report

Faculty

Jonathan H. Chung, MD

Professor of Radiology and Division Chief of Cardiothoracic Imaging

UCSD - University of California San Diego

Tags

Non-infectious Inflammatory

Lungs

Chest CT

Chest

CT

Acquired/Developmental