[CIS-PAGID] panhypogamm in a 22 yo

[Church, Joseph]

Hizentra has much lower IgA than Vivaglobin.  Go with the Hizentra.  JC

 

________________________________

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Marianne Empson
(ADHB)
Sent: Monday, April 04, 2011 2:49 PM
To: 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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