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

Sat Jan 10 12:40:51 EST 2015

Jack,
Since I am speaking for myself and not for Baxter, the age doesn't matter.
This is not a patient that I would treat with HyQvia. Systemic side effects
are low than with IGIV but not lower than IGSC. If this patient is to be
treated, I think hyperfractionating the dose is the best approach.
Richard

On Sat, Jan 10, 2015 at 11:05 AM, Seppänen Mikko <Mikko.Seppanen at hus.fi>
wrote:

> Dear Michael,
>
> a) no serious infections
> b) unspecific hypogammaglobulinemia with double digit responses...
> --->
> I definitely would not treat to start with, and definitely not with these
> side effects and need for immunomodulation due to the side effects caused
> by iatrogenic reasons.
>
> So I completely agree with you!
>
> Side effects like these (and others) are in my experience almost to be
> expected if there are very robust responses. One could argue that
> opsonophagocytic assays were not used, but robust responses at this level
> IMHO suggest Th2-dominant immunity (like Ashish suggests), and IVIg/SCIg
> only serve to activate DC-SIGN and Th2-dominant cytokines even more
> (IL-13,IL-33).
>
> If I recall myself correctly when I was 11, and if I were given a choice
> to choose between recurrent URTIs, CRRS and these side effects, I would
> have chosen the first ones for sure. And this comes from someone with a
> little low IgG (clearly low IgG1 and IgG3), robust responses, asthma,
> allergies and Th2-dominant responses, but absolutely no PIDD...
>
> Mikko Seppänen,
> Helsinki University and Helsinki University Hospital, Finland
>
> ________________________________________
> Lähettäjä: Keller, Michael [MKeller at childrensnational.org]
> Lähetetty: 9. tammikuuta 2015 19:35
> Vastaanottaja: CIS-PIDD
> Aihe: RE: [cis-pidd] Dysgammaglobulinemia and severe IVIG intolerance
>
> Thank you all very much for your responses so far.
>
> I'm told by his previous immunologists that he has fewer infections when
> on IVIG, but its been at the cost of 3-4 days every 3 weeks where he is
> totally incapacitated.
>
>
>
> ________________________________
> From: Kumar, Ashish [Ashish.Kumar at cchmc.org]
> Sent: Friday, January 09, 2015 10:59 AM
> To: CIS-PIDD
> Subject: RE: [cis-pidd] Dysgammaglobulinemia and severe IVIG intolerance
>
> 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
>
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>
> Office: 202.476.5843<tel:202.476.5843>
>
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>
> www.childrensnational.org<http://www.childrensnational.org>
>
>
>
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> --
> Richard L. Wasserman, MD, PhD
> DallasAllergyImmunology
> 7777 Forest Lane, Suite B-332
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