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

Thu Aug 11 13:39:53 EDT 2016

All consistent with NEMO or one of the autosomal recessive versions.

Kate Sullivan, MD PhD
Wallace Chair
Chief of Allergy Immunology
ARC 1216 CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363

On Aug 11, 2016, at 1:09 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

He is short/underweight, but has had chronic feeding issues and IBD so unclear etiology.  I'll ask about sweating.

Megan

-----Original Message-----
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, August 11, 2016 11:04 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] extremely high IgA with immune dysregulation

Sorry..I meant to say IgA.  Is he short?  I have a large family with this...the ectodermal changes can be subtle...but mom can always tell which ones don't sweat.  They are all short however.

james

-----Original Message-----
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Wednesday, August 10, 2016 5:10 PM
To: CIS-PIDD
Subject: RE: [cis-pidd] extremely high IgA with immune dysregulation

I don't recall it being a complaint, but honestly I'm not sure.  He has normal teeth/nails/hair.

Megan

-----Original Message-----
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Wednesday, August 10, 2016 5:01 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] extremely high IgA with immune dysregulation

Megan. I have also seen high IgD in IKBa deficiency. Does the kid sweat?

Sent from my iPhone

On Aug 9, 2016, at 2:07 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org><mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Hi Megan,
In male patients with ID and Hyper-IgA, one has to rule out NEMO. I know the clinical picture is not a NEMO-like one, but the clinical and biological phenotype can be very surprising sometimes in NEMO and I have the rule: Hyper-IgA and immune deficiency = think NEMO !
All the best
Elie

Elie Haddad, MD, PhD,
Professor of Pediatrics, University of Montreal, Head, Pediatric Immunology and Rheumatology Division, CHU Sainte-Justine, 3175 Cote Sainte-Catherine Montreal, QC, H3T 1C5, Canada
Ph: 1 514 345 4713
fax: 1 514 345 4897
e-mail: elie.haddad at umontreal.ca<mailto:elie.haddad at umontreal.ca><mailto:elie.haddad at umontreal.ca>

Le 2016-08-09 ? 10:31, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org><mailto:cis-pidd at lists.clinimmsoc.org>> a ?crit :

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><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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