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

[CIS-PIDD]

Hi Megan:  This might be relevant although this patient doesn't have a h/o IBD.  We are following a nine-year-old girl with a markedly increased IgA (initially at age 6 yrs it was over 2 g/dL, in f/u visits 700-900 mg/dL) and otherwise normal immunoglobulins. She was referred initially by an ENT physician because of really persistent sinusitis (which has continued) and his discovery of her markedly elevated IGA. She has had no other significant infections at all and is and otherwise well grown, developmentally normal, beautiful little girl.  We were wondering whether she might have a defect in the J chain or the poly-Ig receptor but unexpectedly her IgA subclass analysis showed that it was virtually all IgA1 and IEP showed a paraprotein - IgA kappa.  Her flow cytometry has been normal, although B cells have been on the low side. Interestingly, she has had positive EBV PCR (300-400 copies/mL) on 2 of 4 occasions from peripheral blood over the past 4 years. She has had an evaluation by our hematologists including a bone marrow and extensive CT scans without any evidence of malignancy at present, but it is certainly an ominous looking presentation. We are continuing to just watch her carefully for the present.

Have you performed an IEP and looked for EBV in your patient?

Prescott

T. Prescott Atkinson, MD PhD, Professor and Director
Division of Pediatric Allergy, Asthma & Immunology
University of Alabama at Birmingham
Tel: 205-996-9582
Fax: 205-975-7080
Cell: 205-999-7688



From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, August 09, 2016 9:32 AM
To: CIS-PIDD <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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