[PAGID] PAGID Digest, Vol 28, Issue 1

[Jennifer Puck]

Sorry to hear that this patient did not make it. The leg lesions you
describe would not be typical of dominant HIES, which in a 28 year old
should be characterized by history of cutaneous (staph aureus) abscesses,
recurrent pneumonias with pneumatocele formation and high IgE, as well as
typical facies with thickened skin, prominent pores, and increased width
across the bottom of the nose.
Unfortunately fungal disease is a sneaky problem in these patients
(especially with steroids on board), and for any type of infection they
characteristially don't feel unwell or seek medical attention for an acute
episode until it has reached a florid stage.
Jennifer Puck, MD
PS--not sure whether this fits your patient, but we reported a family with
multiple sibs who had high IgE, recurrent infections, cutaneous vasculitis
(especially on the ankles and feet) and myoclonus and cognitive difficulties
( Hay BN, Martin JE, Karp B, Davis J, Darnell D, Solomon B, Turner M,
Holland SM, Puck JM. Familial immunodeficiency with cutaneous vasculitis,
myoclonus, and cognitive impairment. Am J Med Genet A. 2004 125:145-51.) JP



On 9/15/07 7:15 PM, "pagid-request at list.clinimmsoc.org"
<pagid-request at list.clinimmsoc.org> wrote:


> Send PAGID mailing list submissions to

> pagid at list.clinimmsoc.org

> 

> To subscribe or unsubscribe via the World Wide Web, visit

> http://seven.pairlist.net/mailman/listinfo/pagid

> or, via email, send a message with subject or body 'help' to

> pagid-request at list.clinimmsoc.org

> 

> You can reach the person managing the list at

> pagid-owner at list.clinimmsoc.org

> 

> When replying, please edit your Subject line so it is more specific

> than "Re: Contents of PAGID digest..."

> 

> 

> Today's Topics:

> 

>    1. Hyper IgE therapy? (Saxon, Andy M.D.)

>    2. Re: Hyper IgE therapy? (Richard L. Wasserman, M.D.,Ph.D.)

>    3. Re: Hyper IgE therapy? (Saxon, Andy M.D.)

>    4. Re: Hyper IgE therapy? (Charlotte Cunningham-Rundles)

>    5. Re: Hyper IgE therapy? (Bodo Grimbacher)

>    6. Re: Hyper IgE therapy? (Saxon, Andy M.D.)

> 

> 

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

> 

> Message: 1

> Date: Thu, 13 Sep 2007 16:23:27 -0700

> From: "Saxon, Andy M.D." <ASaxon at mednet.ucla.edu>

> Subject: [PAGID] Hyper IgE therapy?

> To: "PAGID \(E-mail\)" <pagid at list.clinimmsoc.org>

> Message-ID:

> <97F2A1CF3410B346A85CD4C19B1C484601B86001 at admedmail5.ad.medctr.ucla.edu>

> 

> Content-Type: text/plain; charset="iso-8859-1"

> 

> 

> 

> 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.

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

> 

> 

> 

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

> 

> Message: 2

> Date: Thu, 13 Sep 2007 21:42:09 -0500

> From: "Richard L. Wasserman, M.D.,Ph.D." <richw3 at swbell.net>

> Subject: Re: [PAGID] Hyper IgE therapy?

> To: pagid at list.clinimmsoc.org

> Message-ID: <46E9F501.8070303 at swbell.net>

> Content-Type: text/plain; charset=ISO-8859-1; format=flowed

> 

> My personal experience is that fungal infection has been a common

> terminal event. Steroid use in these patients has been associated with

> fungal infection. I would add impiric anti-fungal therapy and work hard

> to minimize steroids. If there is an identified bacterial pathogen

> passive immunization with IVIG may be helpful.

> Richard Wasserman

> 

> Saxon, Andy M.D. wrote:

> 

>> 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.

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

>> 

>> 

>> 

>>  

>> 

> 

> 

> 

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

> 

> Message: 3

> Date: Thu, 13 Sep 2007 21:01:07 -0700

> From: "Saxon, Andy M.D." <ASaxon at mednet.ucla.edu>

> Subject: Re: [PAGID] Hyper IgE therapy?

> To: <pagid at list.clinimmsoc.org>

> Message-ID:

> <97F2A1CF3410B346A85CD4C19B1C484601B8600A at admedmail5.ad.medctr.ucla.edu>

> 

> Content-Type: text/plain; charset="iso-8859-1"

> 

> Thanks Richard.  

> 

> He may have fungal infection so they do have that covered. They will try to

> reduce his steroids - apparently he has been on enough - long enough that he

> is likely dependent plus they are giving higher doses right now because of DIC

> etc.  I did have them give him IVIg dose after at least sending Ig levels

> since we don't know what his Ig levels have been.

> 

> Andy Saxon, UCLA

> 

>  

> 

> -----Original Message-----

> From: pagid-bounces at list.clinimmsoc.org

> [mailto:pagid-bounces at list.clinimmsoc.org]On Behalf Of Richard L.

> Wasserman, M.D.,Ph.D.

> Sent: Thursday, September 13, 2007 7:42 PM

> To: pagid at list.clinimmsoc.org

> Subject: Re: [PAGID] Hyper IgE therapy?

> 

> 

> My personal experience is that fungal infection has been a common

> terminal event. Steroid use in these patients has been associated with

> fungal infection. I would add impiric anti-fungal therapy and work hard

> to minimize steroids. If there is an identified bacterial pathogen

> passive immunization with IVIG may be helpful.

> Richard Wasserman

> 

> Saxon, Andy M.D. wrote:

> 

>> 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.

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

> 

> 

> 

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

> 

> Message: 4

> Date: Fri, 14 Sep 2007 12:00:02 -0400

> From: Charlotte Cunningham-Rundles

> <charlotte.cunningham-rundles at mssm.edu>

> Subject: Re: [PAGID] Hyper IgE therapy?

> To: pagid at list.clinimmsoc.org

> Message-ID: <a06110421c3105fa69624@[146.203.53.204]>

> Content-Type: text/plain; charset=us-ascii; format=flowed

> 

> 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.

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

>