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

[Richard Wasserman]

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.

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> 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
>
> Office: 202.476.5843
>
> Fax: 202.476.2280
>
> www.childrensnational.org
>
>
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-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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