[CIS PIDD] [cis-pidd] Autoimmune polyglandular syndrome with low immunoglobulins

[Evan Shereck]

That’s one reason why I am confused. He does not have diarrhea and his serum albumin is 4.2. I don’t have a good reason for borderline IgG.

-Evan

From: Church, Joseph [mailto:JChurch at chla.usc.edu]
Sent: Tuesday, November 06, 2012 3:04 PM
To: CIS-PIDD
Subject: RE: [cis-pidd] Autoimmune polyglandular syndrome with low immunoglobulins

Evan:

I didn't think that APECED/APS1 is associated with immune deficiency.  Why is his IgG borderline?  Does he have protein losing via gut (serum albumin?).

Consider giving him a Hib "boost" and a Pneumovax prior to the procedure.

Joe Church
Children's Hospital Los Angeles

From: Evan Shereck [mailto:shereck at ohsu.edu]
Sent: Tuesday, November 06, 2012 2:50 PM
To: CIS-PIDD
Subject: [cis-pidd] Autoimmune polyglandular syndrome with low immunoglobulins

I was wondering if I could have your opinion on a new patient of mine. He is a 12 yo boy who was recently diagnosed with autoimmune polyglandular syndrome type I confirmed by molecular study with compound heterozygote AIRE gene mutations in March 2012.  He has chronic keratoconjunctivitis and is now nearly blind, failure to thrive, hypoparathyroidism, and recurrent HSV stomatitis.  The ophthalmologists are planning a limbic stem cell transplant for each eye and he will be placed on Tac and MMF afterwards. I performed an immune work-up on him pre-immunosuppression. Below are the results:

IgE- 7
IgG- sublcass I- 358
Total IgG- 608
IgA- 72
IgM- 34
Diptheria titer- 1.1
Tetanus titer- 0.4
Haemophilus titer- 0.1
Prevnar- see attached form
Lymphocyte mitogens- nl response to PHA, con A, and pokeweed
Lymphocyte subsets: Decreased numbers of T cells- Decrease in CD3+, CD8+ T cells (total- 128 cells/ul) - Borderline increased percentage of double negative T cells (7.31% of lymphs), Alpha/beta DNTs of 0.63 % of lymphs,  Decreased numbers of B cells (total 124.18 cells/ul)- No monoclonal B cell population identified - Mild increased percentage of polyclonal CD5 positive B cells for age - Minimally decreased percentage of nonswitched memory B cells (IgD+, CD27+) - Decreased numbers of NK cells (total 49.23 cells/ul)


This is the first such kid that I have encountered with this disorder. I was wondering what your thoughts were about the mildly abnormal immune work-up. Any advice you have, would be much appreciated.

Thanks.

-Evan

Evan Shereck, MD
Assistant Professor of Pediatrics, Co-Director of Pediatric Hematology/Oncology Fellowship
Oregon Health & Science University I  3181 SW Sam Jackson Park Rd, Mail Code: CDRCP  I  Portland, OR 97239
• (Office): 503-494-0829  I  (Fax): 503-494-0714
 • (E-mail): shereck at OHSU.edu<mailto:shereck at OHSU.edu>

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