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

Fri Jan 9 14:56:26 EST 2015

Mike,

At least for the Headache, I was successful in using acetazolamide (Diamox) 250 mg to 500 mg BID for 3 to 7 days before the infusions to 3 to 7 days after.  In one patient whose headaches would begin 2-3 days after the infusion at last 2-3 weeks, continued to treat for 2 weeks after the infusion, after the first week trying to reduce dosage from 500 BID to 250 BID then to 250 qD then D/C, tapering to try to prevent post-medication flare.

Terry Harville MD PhD D(ABMLI) D(ABHI)
*Medical Director, Special Immunology Laboratory
*Medical Director, Histocompatibility Laboratory
*Medical Director, Immunogenetics and Transplantation Laboratory
*Specialist in Pediatric Allergy, Asthma, Immunology, Rheumatology,
Autoimmunity, Hematopoietic Stem cell Transplantation for
Immunodeficiencies, and Organ Transplantation Immunology
*Diplomate of the American Board of Medical Laboratory Immunology
- specializing in diagnostic evaluations of immunodeficiencies
*Diplomate of the American Board of Histocompatibility and Immunogenetics
- specializing in pre- and post-transplantation diagnostic evaluations,
disease associations, and platelet transfusion support

Departments of Pathology and Laboratory Services and Pediatrics
University of Arkansas for Medical Sciences
4301 West Markham
Mail Slot #502
Little Rock, AR  72205-7199

Work Phone 1.......................................501.686.7257
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Email:..................................harvilleterryo at uams.edu

Special Immunology Laboratory......................501.364.1804
Histocompatibility Laboratory......................501.686.7257
Immunogenetics and Transplantation Laboratory......501.686.7374

From: Keller, Michael [mailto:MKeller at childrensnational.org]
Sent: Friday, January 9, 2015 9:10 AM
To: CIS-PIDD
Subject: [cis-pidd] Dysgammaglobulinemia and severe IVIG intolerance

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<http://www.childrensnational.org>

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