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

[CIS-PIDD]

Thanks Prescott & Anthony.  We are going to get protein electrophoresis when he comes back to see heme/onc, so I don't know yet.  WAS sequencing was negative and he has normal platelets.

Prescott - we haven't checked for EBV recently, your case is very interesting.

Megan

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, August 09, 2016 12:27 PM
To: CIS-PIDD
Subject: RE: [cis-pidd] extremely high IgA with immune dysregulation

Is that IgA polyclonal? And you sometimes see high IgA in WAS.

Anthony Hayward

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, August 9, 2016 10:32 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: [cis-pidd] extremely high IgA with immune dysregulation

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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