[CIS PIDD] [cis-pidd] IgG Levels dropping in XLA patient

[CIS-PIDD]

I have also had a couple of patients with antibody deficiency (one CVID and on HyperIgM) who could not maintain their serum IgG levels.  This also correlated with chronic lung disease.  Neither one had any evidence of protein loss (normal albumin and calprotectin).  I agree that consumption is the most likely cause and would look carefully at the lungs.


In both cases we went to daily SC IG to try to keep the serum IgG above 500 mg/dL


Lisa


Lisa Kobrynski, MD, MPH
Associate Professor of Pediatrics
Marcus Professor of Immunology
Section, Allergy/Immunology
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From: cis-pidd at lyris.dundee.net <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Monday, June 19, 2017 2:59:33 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] IgG Levels dropping in XLA patient

Hi Ejaz,
In the best-case scenario, your pt is “gaining weight and height” and the IVIG dose if falling short, but I guess this is just wishful thinking…
If the pt is not losing it (as your results suggest) he is likely consuming it, and 3y old lung opacities (despite the pulmonologists opinion) do deserve a new CT scan and a more directed sampling /biopsy for specific diagnosis and therapy. No lymphoproliferative disease? Is Complement normal? Any renal function deterioration? Is the pt BTK mutation proven or agamm with no B cells? Certain forms of agammm/absent B cell cases are very prone to PCP/PJ infections.
Sergio

Sergio D. Rosenzweig, MD, PhD
Immunology Service, DLM, CC, NIH
mailto:srosenzweig at cc.nih.gov

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From: <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Date: Monday, June 19, 2017 at 1:05 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: [cis-pidd] IgG Levels dropping in XLA patient

Dear all
I am managing a 4 years old with XLA. Who has started with chronic cough .Seen by pulmonary who did bronchoscopy which came out normal except some minor growth of H influenza( Fungi, Atypical and Typical TB negative)
He has been treated with several antibiotics including Augmentin, clinda+ cefopodoxime  without any significant response . Finally started on Bactrim with some improvement however was stopped due to urticarial reaction and angioedema  . Pulmonologist does not feel that a CT scan is required at this time
CT scan done prior to diagnosing XLA ( In 2014 ) showed  multiple  opacities( as per history  I don’t have access to that CT
Another significant trend  noted is that he is dropping his pre IVIG  IgG levels . He is on currently on about 700 mg/kg of IVIG however his last 3 IgG levels have been dropped from 832 to 784 and now 634( lowest he ever had)
Stool for  alpha one antitrypsin is negative
Albumin level is 3.9
Stool for Giardia is negative
Gaining weight and height
No fevers except recently after bronch

Any suggestions why is he dropping his IgG level  and any other suggestions for managing his chronic cough

Thanks

Ejaz Yousef MD

Nemours Children Specialty Care

Jacksonville, FL




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