[PAGID] CF and hypogamma

[Junker, Anne]

We have had several patients with this combo, including one who didn't present until around age 20y with agammaglobulinemia! We started him on IVIg but he was an irregular attender. We have not recommended Ig in the others because they had variable hypogam and were able to make specific antibodies.  I agree with Mel's suggestions as to investigations to verify the need for Ig replacement. If I recall, there is only the one case series that reports this association, and this was in an era before there were more adults than children living with CF. Might be time for a new systematic survey of the CF population.   

Anne Junker, MD
Associate Professor, Division of infectious and Immunological Diseases
BC Children's
Vancouver, BC, Canada
4480 Oak St, Vancouver, BC
604-875-3591
ajunker at cw.bc.ca

----- Original Message -----
From: pagid-bounces at list.clinimmsoc.org <pagid-bounces at list.clinimmsoc.org>
To: pagid at list.clinimmsoc.org <pagid at list.clinimmsoc.org>
Sent: Mon Jan 19 19:47:05 2009
Subject: RE: [PAGID] CF and hypogamma

1. Re: assessment of her CF: What is her FEV1 as % predicted for age, and her weight, % predicted for age ? What other organisms besides pseudomonas are frequent in her sputum ?
 
2. re: assessment of her hypogam: Have you measured specific antibodies against Haemophilus influenzae and pneumococcal polysaccharides ?  Has she ever had bacteremia ?  Does she have sinus diisease and/or otitis disproporpionate to her lung disease ?
 
Older literature suggests an inverse correlation between hypogam and CF lung disease (ie- the lower the IgG the better) but it is difficult to know which was cause and which, effect. 
 
 
 
Melvin Berger, M.D., Ph.D.
Adjunct Professor of Pediatrics and Pathology
Case Western Reserve University
phone 216 844 3237
 
Director, Jeffrey Modell Center for Primary Immune Deficiencies
Division of Allergy-Immunology
Rainbow, Babies and Children's Hospital
University Hospitals of Cleveland
RB&C Rm 504, MS 6008B
11100 Euclid Ave.
Cleveland, OH 44106

  _____  

From: pagid-bounces at list.clinimmsoc.org on behalf of Brynn Wainstein
Sent: Mon 1/19/2009 10:20 PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] CF and hypogamma



Dear all, 

I am hoping for some advice regarding a patient of mine. This is a 9 year old girl with DF508 homozygous cystic fibrosis who was found on routine testing to have hypogamma with results as follows:

        28/07/08        08/10/08        18/12/08 
IgG     2.89            3.27            3.41    g/L (6 - 13) 
IgM     0.4             0.39            0.46    g/L (0.4 - 1.6) 
IgA     0.25            0.26            0.27    g/L (0.5 - 3) 

Normal B cell numbers and T cell subsets. Otherwise clinically well with no significant lung infections despite CF (she is colonised with pseudomonas).

We are considering IVIg replacement despite her clinical wellness because of concurrent CF and persistently low Ig levels. But some reports in the literature suggest worse lung function in patients with hypergamma but don't talk specifically about IVIg.

I would appreciate peoples thoughts and advice on whether this girl should receive IVIg. 

Kind Regards, 

Brynn Wainstein. 

Dr Brynn Wainstein 
Paediatric Clinical Immunologist 
Staff Specialist 
Sydney Children's Hospital 
Tel: +61-2-9382-1515 
Fax: +61-2-9382-1580 


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