[CIS PIDD] [cis-pidd] Hypogammaglobulinemia

[CIS-PIDD]

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

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
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