Monday, July 16, 2007

Oh, Histiocyte, oh histiocyte

Why are you, oh, so confusing?
(a la 'oh christmas tree' tune)
Seriously, what is up with the histiocytes?

Not only can they be the regular old histiocytes that phagocytose debris (reactive), they can also be foamy (a la parasitized macrophages), be xanthomatous, be a proliferative disorder (benign OR malignant). There's more of what they can do that's just not coming to mind.

Who ARE these superpower cells? And why do we know so little about them?


Speaking of histiocytes, someone REALLY needs to look at the field and simplify the names. Jeff Callen? Vicky Werth? Anyone??

Look, why is Langerhans cell histicytosis also called Class I histiocytosis or histiocytosis X? Why does it comprise multiple named entities such as Letterer-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma, congenital self-healing reticulohistiocytosis (which is also called Hashimoto-Pritzker disease)? I understand that historically, they've been poorly understood, and that the numerous names reflect the lack of understanding and aggrement regarding their origins. But with the advent of electron microscopy and the development of immunohistochemical stains, we're now pretty sure that they're all related. Perhaps all variations along a theme? Along a spectrum?

I'm sure congestive heart failure probably had some misnomers and different names along the way, while it was being worked out. But once it got worked out, medicine as a field got together to simplify their nomenclature. Now you got just plain old CHF and diastolic CHF.

So now all we need is SOMEONE to stand up and say, enough with all these ridiculous names. Let's consolidate what we know today, lose the dead weight, and move the field ahead. Lump the problems and how to think about them into ways that make clinical sense.

Let's be lumpers, not splitters. I'm sure Letterer, Siwe, Hand, Schuller, Christian, Hashimoto, and Pritzker don't mind anymore...

photo credit electron microscopy of the classic Birbeck granules


Anonymous Don Johnson said...

Congrats! You sound far more informed on Langerhans Cell Histiocytosis then most physicians.

I wanted to point out a resource to you and your peers. I just posted a video series about Langerhans Cell Histiocytosis on YouTube. Links and brief descriptions for each of the 7 nine-minute videos can be found here:

Part 2 covers all the different historical and outdated names (histiocytosis X, Letter-Siwe, etc.) that you were frustrated by in your most recent post. The other 6 parts cover many other details of the disease and its treatment. The series is presented by Dr. Kenneth McClain from Texas Children's Cancer Center, one of the world's leading researchers on LCH. He also happens to treat our son for LCH.

A number of LCH researchers have stated that it has been challenging to get the dermatology physicians to take LCH seriously, and to treat it accordingly.

I'm all for moving the field forward! I hope you can make some progress with the dermatology field and your peers.

Thanks for taking an interest in LCH.

8:33 AM  
Blogger wandering visitor said...

Hi DJ,
thanks for your post and the link.

Am not sure which dermatologists you are referring to. At the place where I am training, LCH, if it involves organs other than the skin and interferes with function of those organs, is taken quite seriously.

The post was meant to highlight that it's hard to understand the concepts when one first starts out, and that, in general, dermatology nomenclature could use some simplification. I don't think established people in the field would belittle multisystem/progressive LCH.

6:59 PM  

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