[CIS-PAGID] panhypogamm in a 22 yo

[Cunningham-Rundles, Charlotte]

I¹d go with SQ too.



Charlotte Cunningham-Rundles MD PhD
Departments of Medicine, Pediatrics
The Immunology Institute
Mount Sinai School of Medicine
1425 Madison Avenue,
New York City, New York, 10029
212 659 9268 (phone)
212 987 5593 (fax)



From: "Marianne Empson (ADHB)" <MarianneE at adhb.govt.nz>
Reply-To: PAGID <pagid at list.clinimmsoc.org>
Date: Tue, 5 Apr 2011 09:48:42 +1200
To: PAGID <pagid at list.clinimmsoc.org>
Subject: Re: [CIS-PAGID] panhypogamm in a 22 yo

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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