[CIS PIDD] [cis-pidd] Hypogammaglobulinemia

Tue Sep 26 14:20:21 EDT 2017

Dr. Shereck:

A few questions/suggestions –

(1) has the child been evaluated for chronic infections?  EBV, CMV, HTLV, HIV, and mycobacteria (both TB and non) would be on my D/Dx … and if she had any exotic exposures (animals, forest/farm, recreational water), the list would get longer.  Specific IgG could certainly have a transient activity against some occult viral infection … and even some bacterial infections.

(2) has she had an evaluation of antiviral titers pre-IVIG initiation?

(3) has the abdominal pain been evaluated?  If she’s thin enough, superior mesenteric artery (Wilkie’s) syndrome would be part of my D/Dx.

I think most people would agree that re-starting IVIG would be a prudent route.  The question is what are we treating / missing.

Good luck with the case.

Karl

Karl O. A. Yu, M.D., Ph.D., F.A.A.P.
Scientist II and Assistant Director, Center for Infectious Diseases and Immunology
RGH Research Institute | Rochester General Hospital | Rochester Regional Health
1425 Portland Ave., Room R-403, Rochester, NY   14621
Tel  585-922-3709  |  Fax  585-922-2415

From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Tuesday, September 26, 2017 2:04 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Hypogammaglobulinemia

Yes, both were done and were normal.

-Evan
_____________________
On Tue, Sep 26, 2017 at 10:54 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:
If not done, fecal alpha-1-antitrypsin and calprotectin may be helpful.

Joe Church
Children’s Hospital Los Angeles
_____________________
From: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> [mailto:cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>] On Behalf Of CIS-PIDD
Sent: Tuesday, September 26, 2017 10:34 AM
To: CIS-PIDD
Subject: [cis-pidd] Hypogammaglobulinemia (EXTERNAL EMAIL)

All,

I am seeking advice about a 10 year old female with low IgG. who first came to my attention 3 years ago. At that time, she had low energy, abdominal pain and low-grade fevers (99.9-100.4). She also has asthma. There were no obvious findings on physical exam other than a skinny, sad, child.

Her immune work-up revealed an IgG level of 260 with normal IgM and IgA levels. She has vaccine responses to strep pneumo, tetanus, and diphtheria. She had a normal total B cell count with switch to memory cells.

She was started on IVIG and her symptoms disappeared right away. She had to be maintained on IVIG Q2 weeks to keep her level > 600 to keep her asymptomatic. Her teachers even commented on how much better she was at school after starting the IVIG.

Last year, the family decided to come off the IVIG. Again her symptoms have returned. She is missing 2-3 days of school/week. She is having abdominal pain daily. She has no energy and feels the need to nap all the time (even at school).

Repeat immune testing is consistent with her previous testing with the low IgG being the only abnormality. In addition, she had B-cell immunophenotyping which was normal. Inflammatory markers (ESR and CRP were normal). CBC is normal. She does have a mildly low albumin that fluctuates from 2.9-3.

She has had an endoscopy, colonoscopy which were normal. Stool studies are normal. Urine micro and  urinalysis were normal.

It does not seem like a primary production issue. So, is she losing her IgG somewhere and if so, where? Where else should I be looking?

In the meantime, she is going to re-start her IVIG because it does seem to make a big difference in her overall health and quality of life.

Thank you for your thoughts.

-Evan

Evan Shereck, MD, MEd
Associate Professor of Pediatrics
Director, Pediatric Hematology/Oncology Fellowship
Block Co-Director, Blood and Host Defense
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