[CIS-PAGID] Hypogam following Fontan

[Church, Joseph]

Thank you, Tom and Gigi.

 

The patient's IgM and A are normal (60 and 44mg/dL, respectively).  She
has pan-T lymphopenia:

            CD3                28% (367)

            CD4                11% (150)

            CD8                12% (162)

            CD19              34% (150)

            CD16/56        23% (162).

 

Joe Church

 

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Fleisher, Thomas
(NIH/CC/DLM) [E]
Sent: Thursday, December 01, 2011 7:33 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Hypogam following Fontan

 

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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