[CIS PIDD] 9 yo with CVID-like picture with persistent low IgG on IVIG

[Sergio Rosenzweig]

Hi Shayma,
What about his albumin level, alpha 1 antitrysin clearance and 24h urine
collection? No previous cardiac surgery? As you suggest, more frequent
infusions or Sc route would work better that keep on increasing the IVIG
dose: at some point all his FcRn would be saturated and and he will be
pushing towards a FcgammaR/MBL degradation pathway, rather than the FcRn
(IgG half-life protective) pathway. Did you check for any of these
receptors?
Sergio

Sergio D. Rosenzweig, MD, PhD
Chief, Infectious Diseases Susceptibility Unit
Laboratory of Host Defenses, NIAID, NIH
10 Center Dr., Bldg. 10, CRC 5W-3888
Bethesda, MD 20892-1456
Phone (301) 451 8971
Fax (301) 451 7901
Cell (240) 361 7617
Pager 102 10678
srosenzweig at niaid.nih.gov
 
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>>> Shayna Burke  06/14/12 10:06 AM >>>

Colleagues,
We have a 9 yo boy with Down Syndrome that was diagnosed with
hypogam/CVID like picture a little over a year ago, whom we cannot get
IgG levels above 200s.  He has a history of recurrent pneumonias and
sinusitis and was found originally to have the following labs:
IgA 74, IgM 32, and IgG 241 (nl 580-1256), and had 1/14 protective
pneumococcal serotypes. 
He was given pneumovax and 2 months later his labs showed:
IgA 62, IgM <25, IgG216.
Tetanus was protective at 0.6 and diphtheria was also at 0.05.  He
continued to only have 1/14 protective pneumococcal titers.
He was started on Gammagaurd liquid at 500mg/kg/dose in March 2011 and
has gotten IVIG every 28 days since then.  However, we have not been
able to get his trough out of the 200s (ie the troughs have been 203,
252, 203, and 278).  We have increased his dose due to these troughs and
he is now up to 1.25grams/kg/dose and this resulted in the most recent
IgG trough of 278 (also now has normal IgA and IgM).  Along with this
most recent trough, we did obtain titers to see if he is maintaining any
protection from the IVIG, but he had no protection to Varicella (IgG
negative) and 0/14 protective pneumococcal sterotypes.  He does not have
diarrhea, and actually has constipation that requires a laxative every
other day.  He is not edematous.  The family is not interested in SCIG
or coming in more frequently for infusions.  Clinically, he is doing
well and has not had any pneumonias since starting IVIG .
Questions for the group:
1)Any suggestions on if we should continue to push the dose or back down
due to possible inhibition of B cells at this high of doses?   What
would you recommend we do for this patient?
2)Any suggestions of possible etiologies for where his IgG is going or
why we cannot get his trough up?
Thank you very much for your help.
Sincerely,
Shayna Burke, MD
Fellow, Allergy/Immunology
Children’s Hospital of Pittsburgh
 

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