[CIS-PAGID] Hypogam following Fontan

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

Joe,
Thanks, I think that the fact that the IgM and IgA along with the IgG being significantly below the lower limit for age is consistent with lymphatic loss as are the lymphocyte findings (B and NK relatively normal). I would predict that if you evaluated CD45RA/RO you will find that the majority of her CD4 cells are CD45RO positive as the lymphatic circulation is predominated by naïve T cells. I question whether you would accomplish much with replacement and suggest you go back to some of the older papers by Warren Strober and Tom Waldmann (JCI 46:1643, 1967) if you can find them, describing their experience in intestinal lymphangiectasia which is at some level what this represents. In addition, thoracic duct drainage was used in the past as a form of immunosuppression and that data might also help guide therapeutic decisions regarding the need to consider whether treatment to prevent infection is indicated.
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 10:41 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Hypogam following Fontan

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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