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

[Keller, Michael]

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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