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

[CIS-PIDD]

Monoclonal, oligoclonal or polyclonal?
Bodo
****************************************
Univ.-Prof. Dr. med. B. Grimbacher

Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Breisacherstraße 115, 79106 Freiburg
bodo.grimbacher at uniklinik-freiburg.de
www.uniklinik-freiburg.de/cci

Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Datum: Wednesday 10 August 2016 13:10
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Betreff: Re: [cis-pidd] extremely high IgA with immune dysregulation

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<mailto: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<mailto: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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