[CIS PIDD] [cis-pidd] Dysgammaglobulinemia and severe IVIG intolerance

Fri Jan 9 10:59:20 EST 2015

Is he actually better with IVIG supplementation? If he can make antibodies to polysaccharide antigens (not PCV7 or PCV13), has normal IgA, are we just fixing a number? I would explore other reasons/treatments for recurrent respiratory illness i.e. nasal steroids.

Ashish Kumar, MD, PhD
Cancer and Blood Diseases Institute
Division of Bone Marrow Transplantation and Immune Deficiency
Cincinnati Children's Hospital Medical Center
http://www.cincinnatichildrens.org/bio/k/ashish-kumar/
http://www.cincinnatichildrens.org/research/divisions/b/bone-marrow/labs/kumar/default/

From: Bleesing, Jacob [mailto:Jack.Bleesing at cchmc.org]
Sent: Friday, January 09, 2015 10:37 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] Dysgammaglobulinemia and severe IVIG intolerance

Richard: having been involved in the HYQVIA studies (and assuming for a moment that this particular patient is an adult - in the eyes of the FDA), can you put this scenario in the context of HYQVIA.

Thanks!

Jack

________________________________
From: Richard Wasserman [drrichwasserman at gmail.com]
Sent: Friday, January 09, 2015 10:19 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] Dysgammaglobulinemia and severe IVIG intolerance
It would be reasonable to manage him with antibiotic prophylaxis alone, at least for several months to let the memory of IgG side effects fade. I doubt that it will be an adequate long term solution. When you are ready to try IgG again, I would use Gammagard by subq push. I would give 1gram/day for as many days per week as it took to achieve your desired dose. See Pediatr Allergy Immunol. 2013 Feb;24(1):49-53. doi: 10.1111/pai.12026.<UrlBlockedError.aspx>

This approach has almost always been successful. I would not give steroids regularly under any circumstances.

Good luck,
Richard Wasserman
Dallas

On Fri, Jan 9, 2015 at 9:09 AM, Keller, Michael <MKeller at childrensnational.org<mailto:MKeller at childrensnational.org>> wrote:
Hello all,

I would be most grateful for thoughts on a very challenging patient who I recently saw.

11 yr old boy with multi-year history of hypogammaglobulnemia (down to 300s) and recurrent sinopulmonary infections (though none severe, and nearly all respond well to outpatient antibiotics).  He has normal lymphocyte flow cytometry, and most outside records that I see show intact vaccine responses (including double digit responses to most pneumovax serotypes).

After meeting him last month, his labs were as follows:
IgG 904 (on IVIG),
IgA 52
IgM 126
IgE 66
Abs CD3 2299 cell/uL
CD3/4 1256
CD3/8 858
CD16/56: 157
CD19 539, of which 8% are CD27/IgD-.
CD4/CD45RA to CD4/CD45RO ratio of 3:1
TCR g/d: 8%
SAP flow cytometry: 96% of CD8 cells, 91% of NKT cells

He was initiated on IVIG 3 years ago, and has had awful adverse reactions both during and after infusions (body ache, severe headache, vomiting).  This has persisted despite trying multiple different products (Carimune, Privigen, Gammagard) and multiple pre- and post-infusion medications (steroids, IV fluids, benadryl, NSAIDs).   The only thing that seems to lessen his post-infusion symptoms are a steroid taper.  Triptans had no effect.  He is now also seeing a pain specialist and has been on opiates for some of these headaches.

He tried hizentra in the past and had a reported reaction during the infusion (dizziness, pain) which necessitated an ER trip.  However he was not documented to have anaphylaxis.   The family are unwilling at this point to try sub-q again.

I'm wondering:
1) Does this child truly need IVIG at this point?  I'm tempted to trial him off and give prophylactic antibiotics a try.
2) Are there any other good strategies for preventing (what I suspect is) aseptic meningitis?  Using high dose oral steroids every 3 weeks seems awfully risky to me.

Thanks very much,
Mike

-------------------------

Michael D. Keller MD

Assistant Professor, Division of Allergy / Immunology

Center for Cancer and Immunology Research

Children's National Health System

111 Michigan Ave NW, Room 1W-314B

Washington, DC 20010

Clinic: 202.476.3016<tel:202.476.3016>

Office: 202.476.5843<tel:202.476.5843>

Fax: 202.476.2280<tel:202.476.2280>

www.childrensnational.org<http://www.childrensnational.org>

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