[CIS-PAGID] Hypogam following Fontan

[Fleisher, Thomas (NIH/CC/DLM) [E]]

Joe,
One of the findings in IL is that the decrease in the different classes of Igs vary, ie shorter half life Igs (ie IgM) have somewhat less of a decrease compared to the longer half life Igs (ie IgG). So I would consider the discrepancy between IgG and albumen a bit extreme but still plausible with lymphatic loss. What is the IgM level and the lymphocyte count? The majority of congenital IL patients do not have infectious problems despite their low Igs and lymphocyte counts.
Tom


Thomas A. Fleisher, M.D.
Chief, Department of Laboratory Medicine
NIH Clinical Center
301 496-5668 (T)
301 402-1612 (F)
From: Church, Joseph [mailto:JChurch at chla.usc.edu]
Sent: Thursday, December 01, 2011 10:26 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Hypogam following Fontan

No, Andy.

The hypogam is thought to be mechanical secibdary to increased pressure in the GI lymphatics due to the specific surgical procedure.  Patients with this process (post-Fontan hypogam+hypoalbuminemia) who go on to require heart transplant normalize Ig levels and even with post-transplant immunosuppression often have normal antibody responses.

Joe Church

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Saxon, Andy M.D.
Sent: Thursday, December 01, 2011 7:21 AM
To: 'pagid at list.clinimmsoc.org'
Subject: Re: [CIS-PAGID] Hypogam following Fontan

Hi Joe: Let me show my lack of pediatric knowledge. Is there recognized thymic developmental issues associated the hypoplastic left heart syndrome as there are in the combined cardiac/thymic issues seen in deGeorge?

Andy Saxon, MD
UCLA

________________________________
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Church, Joseph
Sent: Thursday, December 01, 2011 7:17 AM
To: pagid at list.clinimmsoc.org
Subject: [CIS-PAGID] Hypogam following Fontan
Colleagues:

I am seeing a 7yo girl with hypoplastic left heart syndrome s/p Fontan procedure.

As many of these patients she has developed progressively decreasing IgG.  Her tetanus, Hib and pneumococcal antibodies remain protective, and she has had no serious infections.  She also has T-lymphopenia.

Of particular interest is that her low IgG (currently 155 mg/dL) seems out of proportion to her hypoalbuminemia (2.9-3.2 mg/dL).

Has anyone seen this combination of findings?

I am leaning toward beginning Ig replacement because of the progressive decrease in IgG levels.

Thanks for your help.

Joe Church
Children's Hospital Los Angeles






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