[PAGID] Hyper IgE therapy?

Bodo Grimbacher bodo.grimbacher at uniklinik-freiburg.de
Sat Sep 15 16:23:51 EDT 2007



> 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

>>

>>

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