[PAGID] CVID question

[Saxon, Andy M.D.]

I wonder if these subjects represent a subtype of CVID. Once you rule out
BTK disorders, e.g. normal number of peripheral B cells, is there an
investigation the group should do to see if these CVID patients with "absent
Ig which includes an IgG of <100" are have a common phenotype? and possibly
even genotype? Nature of T cells (RA vs RO), markers on B cells, TACI
mutations etc.?  

Could the group come up with a protocal to study these people

Andy Saxon

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org]On Behalf Of dmvascon at usp.br
Sent: Saturday, July 29, 2006 6:50 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] CVID question


We have seen some patients with very low IgG (less than 100 mg/dL) and
undetectable IgA, IgM and IgE, and one patient with undetectable IgG(< 40
mg/dL), IgA and IgE but normal IgM (~50 mg/dL). 
This latter patient presented a history of respiratory infections with few
digestive symptoms.

All the best,

Dewton

Dewton de Moraes Vasconcelos, M.D., Ph.D.
Primary Immunodeficiencies Outpatient Unit - ADEE3003
Department of Dermatology
University of São Paulo Medical School



Citando "Kathleen E. Sullivan" <sullivak at mail.med.upenn.edu>:

> We have a teenager who presented in that manner and did not have an  
> extensive infection history.
> 
> Kathleen E. Sullivan MD PhD
> Chief, Division of Allergy and Immunology
> Associate Professor of Pediatrics
> The Children's Hospital of Philadelphia
> (p) 215-590-1697
> (f) 215-590-3044
> 
> 
> On Jul 28, 2006, at 6:36 PM, Riedl, Marc wrote:
> 
> > We've recently seen a 41yo gentleman s/p pneumonia and sepsis found  
> > to have
> > undetectable IgG, IgA, IgM or IgE which has been confirmed by repeat
> > testing.  He has normal B/T cell numbers. Neither Dr. Andy Saxon or  
> > I have
> > seen a CVI patient with such profound panhypogammaglobulinemia and  
> > we were
> > curious if others have seen CVI patients with absolutely no detectable
> > antibody of any isotype by routine assays.
> >
> > Best,
> >
> > Marc
> >
> > Marc Riedl, M.D., M.S.
> > Assistant Professor of Medicine
> > Clinical Immunology and Allergy
> > UCLA - David Geffen School of Medicine
> > 10833 Le Conte Ave, 52-175 CHS
> > Los Angeles, CA  90095-1680
> >
> >
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