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

[John Ziegler]

Dear Michelle

About 5% or more of the population are genetically non-responsive to HBV vaccination so I wouldn't make much of that.  (The patient should be counselled that he is not protected against HBV and he should be evaluated for HBV infection, HBsAg etc.)

Apart from IgA deficiency there is no laboratory evidence so far of a host defence defect. It would be appropriate to evaluate further for SAD before you commence a trial of IVIG, although the isoaggs suggest not. I would also do NBT or DHR. No doubt the chest people will look at ciliary function and do a sweat test.

Best wishes

John



Professor John B. Ziegler, AM
Department of Immunology & Infectious Diseases
Sydney Children's Hospital
High St., Randwick NSW 2031
Australia
T: (02) 93821515
F: + 61 + 2 93821580
E: j.ziegler at unsw.edu.au<mailto:j.ziegler at unsw.edu.au>





From: Michelle Halbrich [mailto:michelle.halbrich at hotmail.com]
Sent: Monday, 7 July 2014 9:47 PM
To: CIS-PIDD
Subject: RE: VS: [cis-pidd] Recurrent infection, no IgA, high IgM, normal IgG

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
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