[CIS PIDD] [cis-pidd] extremely high IgA with immune dysregulation

[CIS-PIDD]

Hi Megan,
I had one patient with hyperIgD syndrome who also had very high IgA (in
2000's).
IgA nephropathy, hepatitis, and HIV can also go with high IgA levels.
Best,
Yeshim


Yeshim Yilmaz Demirdag
Assistant Professor of Pediatrics
Columbia University Medical Center
New York
phone: (212) 305 2300
fax: (212) 305 4538


On Tue, Aug 9, 2016 at 10:31 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Hi Everyone,
>
>
>
> I have a 3yo male that I have followed for suspected immune dysregulation
> syndrome, with early-onset IBD and a history of recurrent infections.  His
> infections have actually improved, and his IBD is responding to therapy.  A
> very extensive immunology evaluation that I won’t detail here has been
> essentially normal with the exception of mildly increased CD4 memory
> cells.  He has persistently had hypergammaglobulinemia.  We have been
> unable to obtain clinical exome, a clinical targeted early-onset IBD
> sequencing panel was negative.
>
>
>
> My question for the group – his IgA is now very elevated (>2x his IgG
> level). He is receiving steroids and Imuran.
>
>
>
> 2015 – IgG 1650, IgA 395, IgM 99; CD19 count 4700
>
> 2016 – IgG 2500, *IgA >6000*, IgM 165; CD19 count 300
>
>
>
> I’ve never seen IgA this high in a child.  Has anyone seen this in IBD
> (treated and controlled) or a primary immunodeficiency?  His sequencing
> panel included IL10RA, but not IL10, this seems much higher than that
> reporter.
>
>
>
> A bone marrow last year did show increased plasma cells and heme/onc is
> going to see him again.  A plasma cell disorder seems very unlikely in this
> age group, but you never know.
>
>
>
>
>
> Thanks,
>
>
>
> Megan
>
>
>
>
>
> Megan A. Cooper, MD, PhD
>
> Assistant Professor, Department of Pediatrics
>
> Division of Rheumatology
>
> Washington University School of Medicine
>
> Cooper_m at kids.wustl.edu
>
> Lab website: http://research.peds.wustl.edu/Default.aspx?alias=
> research.peds.wustl.edu/Labs/Cooper_M
>
>
>
>
>
>
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