[CIS-PAGID] panhypogamm in a 22 yo

[Marianne Empson (ADHB)]

I have a patient with CVID who started to have systemic reactions to IVIg and was found to have very high anti-IgA antibodies.  Her reactions persisted despite manipulating IV rate, frequency and dose per infusion as well as premed but the features changed from severe chest pain and rigors to severe opiate requiring headaches with every infusion  She has now been on subQ Ig for a year with absolutely no problems.

I would therefore try subQ replacement therapy.

Marianne Empson
Clinical immunologist
Auckland
NZ
________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] On Behalf Of Hare, Nathaniel D [NHare at Cheshire-Med.COM]
Sent: Tuesday, 5 April 2011 7:52 a.m.
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] panhypogamm in a 22 yo

He should be tested for X-linked agammaglobulinemia.


Nathaniel D. Hare MD
Allergy & Immunology
CMC - Dartmouth Hitchcock Keene
Keene, NH  03431

ph  (603) 354-5496
fax (603) 354-5498
________________________________
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Nancy Kingston
Sent: Monday, April 04, 2011 3:37 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] panhypogamm in a 22 yo

Dear Colleagues,
An interesting patient was recently referred to me from an oncology colleague who had been seeing him for autoimmune neutropenia and thrombocytopenia.  He had also treated him for panhypogammaglobulinemia with Gammagard.  He was having anaphylaxis to the product and so replacement IgG was stopped.He had been previously dx'd with CVID.

The patient's chief complaint is not infections at all.  He comes with a history mainly of autoimmune cytopenias, on Mepron, Cellcept, and prednisone.  He also has diarrhea fatigue and a 60 pound weight loss in the last couple of years.  CBC/diff is currently ok. No endocrine issues or rashes. The patient and his family had previously been advised to not get primary immunizations.

No family hx of immune defect or consanguinity

Has had mild splenomegaly, no hepatomegaly.
He has had his tonsils and appendix removed.

Recent labs:
IgG 74-77 (578-1228 mg/dl)
IgA <6 (59-337 mg/dl)
IgM <4-9 (23-166 mg/dl)
IgE <4 (0-150 mg/dl)
Anti-IgA Ab IgG-377.6 (<52 U neg)
CD3, CD4, CD8, CD19 total numbers are WNL
CD16 abs # 23 (24-911)
Decreased NK cell function by the Cincinnati lab
No antibody titers for H. influenza, tetanus, 23 serotypes of S. pneumonia, or EBV.
Total complement is normal.
Hx of Negative ALPS testing.

I would appreciate any further opinions you might share regarding gammaglobulin replacement and/or testing for this patient.
Thank you for your input.
Nancy Wasserbauer, DO
Akron Children's Hospital Dept of Allergy and Immunology



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