[PAGID] Hyper IgE therapy?

[Saxon, Andy M.D.]

Thanks for the input on BMT. Unfortunately he died from overwhelming sepsis with DIC the next day  I have not tracked down the proof he actually had HIES much less if  he had a Stat3 mutation  On checking what I could, he carried diagnosis of HIES with an IgE of 10,000 IU/mL but his infections were almost all localized to his lower extremities and consisted of severe burrowing ulcers. He did not have a history of pulmonary or bone or internal "abcesses" as best I know at this point.  His legs did not look like pyoderma gangranosa but more like hydradenitis supppertiva (which is clearly was not).  

I hope to get more information from the immunology doctor who took care of him as a youngster before he was lost to them. 

Andy Saxon, UCLA


-----Original Message-----
From:	pagid-bounces at list.clinimmsoc.org on behalf of Bodo Grimbacher
Sent:	Sat 9/15/2007 1:23 PM
To:	pagid at list.clinimmsoc.org; Holland, Steven (NIH/NIAID) [E]
Cc:	
Subject:	Re: [PAGID] Hyper IgE therapy?


> Andy, 



> If this is a STAT3 mutated HIES (Job syndrome) then BMT should be 

> considered if well enough.

Is he receiving steroids as part of sepsis tx?
STAT3, among other cytokines, controls IL22 and IL17...
Steve, what do you think?
Bodo


> we have used WBC infusions in this scenario ( brain abscess in that 

> case) and I know the NIH has as well.

>

>  I don't   know if G(M) -CSF has been used, but we have used  GM-CSF 

> in CGD with terrible GI disease which helped avoid colostomy

>

>

>> We have just received an very ill 28 yo man with alleged Hyper IgE ID 

>> who is septic, dic etc. My question is:

>>

>> "Is there any immune directive intervention that is useful in this 

>> disease in general?"

>> He is receiving excellent agressive regular medical management and 

>> unfortunately is on a necessary dose of steroids. I'm not aware of 

>> any proven cytokine, hematopoetic factor etc that has been shown to 

>> improve the underlying immune problem.

>>

>> You rapid input even to say nothing new is out there would be greatly 

>> appreciated.

>>

>> Andy Saxon, MD

>> UCLA

>>

>>

>> ----------------------------------------------------------

>> IMPORTANT WARNING:  This email (and any attachments) is only intended 

>> for the use of the person or entity to which it is addressed, and may 

>> contain information that is privileged and confidential.  You, the 

>> recipient, are obligated to maintain it in a safe, secure and 

>> confidential manner.  Unauthorized redisclosure or failure to 

>> maintain confidentiality may subject you to federal and state 

>> penalties. If you are not the recipient, please immediately notify us 

>> by return email, and delete this message from your computer.

>> ----------------------------------------------------------

>

>






----------------------------------------------------------
IMPORTANT WARNING:  This email (and any attachments) is only intended for the use of the person or entity to which it is addressed, and may contain information that is privileged and confidential.  You, the recipient, are obligated to maintain it in a safe, secure and confidential manner.  Unauthorized redisclosure or failure to maintain confidentiality may subject you to federal and state penalties. If you are not the recipient, please immediately notify us by return email, and delete this message from your computer.
----------------------------------------------------------

-------------- next part --------------
A non-text attachment was scrubbed...
Name: not available
Type: application/ms-tnef
Size: 4443 bytes
Desc: not available
Url : <http://seven.pairlist.net/mailman/private/pagid/attachments/20070915/d9399ccd/attachment-0001.bin>