[CIS PIDD] [cis-pidd] Recurrent infection, no IgA, high IgM, normal IgG

[Michelle Halbrich]

Hi everyone,
 
I was wondering if I could ask your thoughts on a patient I saw. I would appreciate additional suggestions:

27 year old male
with a history of IgA deficiency, presented prior to dental surgery for the
“go-ahead” from Immunology for the surgery.



History is
significant for “18” T-tube surgeries for recurrent OM, 4 episodes of
pneumonia, 2 sinus infections, gastroenteritis around once a year (when
everyone else is sick), a fungal skin infection 2 years ago treated with
creams, a dental abscess (reason for the dental surgery), 2-3 UTIs.


He has a history
of “asthma” diagnosed at the age of 6, difficult to treat, on multiple puffers.
He has symptoms of AR.


He was vaccinated
for Hep B, but required a booster because “it did not take”.


Family history is
significant for the maternal grandfather who had 4 malignancies, including
vocal cancer.


He is a singer.


I had ordered
vaccine serology as part of the initial workup, and when he was not protected
against MMR, his family MD vaccinated him (he did fine after receiving the live
vaccine).


In January 2014,
he was in an MVA. He was started on prophylactic antibiotics while awaiting workup
(and referral to the immunology centre). May 2014 he was admitted for a few
days with pneumonia.


Labs: this is
what I have:


-
Low IgA, high-ish normal IgG, and elevated IgM on multiple samples: IgA
< 0.1, IgG 14.9, IgM 5.4


- WBC 3.8, low neutrophils of 1.39, otherwise unremarkable CBC; normal
lymphocyte phenotyping with CD19 count of 140, CD8 6178, NK 110;
isohaemagluttinin anti-A titre 1:8, anti-B titre 1:128; normal PHA lymphocyte
stimulation assay of 614.


- normal total protein and albumin


- Diphtheria serology 0.34,0.29 IU/mL, Tetanus serology 0.22,0.17 IU/mL
(done twice). 


- Non-reactive Hep B (0.54mIU/mL) despite vaccination; non-reactive
measles (129.54 mIU/L), mumps (15.03 RU/mL), intermediate rubella (5.1 IU/mL),
that responded to vaccination (measles 910.95 mIU/mL, mumps 92.69 RU/mL,
rubella 7.1 IU/mL). 


- SPEP shows diffuse polyclonal increase in gamma globulins, increased
alpha 1 and alpha 2 (consistent with acute phase reaction), NOT suggestive of a
monoclonal pattern. 


- HIV antigen and antibody negative. 


- CT chest, Jan 2014: diffuse bronchial wall thickening, no bronchial
dilation, numerous peribronchovascular ill-defined nodular opacities and
branching opacities likely related to inflammatory or infectious processes.


- Abdominal Ultrasound: normal


- Spine MRI, May 2014: Schmorl nodes involving T11-T12, T12-L1, L1-L2,
mild disc desiccation at L1-L2 and L2-L3, minimal dorsal disc bulge at L4-L5,
L5-S1, with no evidence of disc herniation


Any suggestions?
I suspect he needs Ig replacement, despite the response to the MMR vaccine.
What about CD40? CD40L? I have referred him to Respirology and the appointment
is pending.


Thank you!
 
 
Michelle Halbrich, MD, FRCPC
Paediatrician, Clinical Immunology and Allergy
Toronto, Canada
 		 	   		  
---
The CIS-PIDD listserv is supported by the Clinical Immunology Society
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=45124359
or send a blank email to leave-45124359-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/pipermail/pagid/attachments/20140707/bac0ebc8/attachment-0001.html>