[CIS-PAGID] Hypogam following Fontan

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

Sorry, I failed to read the entire message so the issue to be excluded seems to be did the surgery involve enough to have any possibility of impacting the thoracic duct versus does she have a lymphoma which as noted in an abstract with the earlier email since this has been described (an extremely rare secondary cause of IL). Seems like constrictive pericarditis is unlikely as are primary IL or the inflammatory secondary IL. 

Thomas A. Fleisher, M.D.
Chief, Department of Laboratory Medicine
NIH Clinical Center
301 496-5668 (T)
301 402-1612 (F)

-----Original Message-----
From: Dowling, Paul MD [mailto:pdowling at cmh.edu] 
Sent: Friday, December 16, 2011 2:52 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Hypogam following Fontan

Dear Colleagues:

I have an interesting case related to the previous one described above concerning protein/antibody loss.
Previously healthy 10 year old female presenting with acute R side abdominal swelling. Imaging identified a large right sided plerual effusion that was immediately drained (almost 1000mL) with chest tube placed soon after. No source for the effusion could be identified as child has been healthy. Cytology on the pleural fluid was largely lymphocytic (97%), gram stain negative without any organism or suspicion for infectious orgin. All cutures including blood and pleural fluid have been negative to date.

Child continues to drain massive amounts of chylous material from the chest tube, exceeding 6,000mL in just under 72 hours. With this, we have seen her lymphocytes fall over time to an ALC of 900 this morning. Total serum protein 4.7 g/dL and albumin 2.4.  Pleural effusion protein was 575mg/dL. Light's criteria would indicate this as more of an exudative effusion by calculation. Flow cytometry revealed pan lymphopenia with a CD4/CD8 ratio of nearly 1, with preservation of B and NK cell lines. We are concerned because her IgG is falling as well. Currently, 430 yesterday and today now 400.  We are assuming this is from the lymphatic fluid loss from the effusion but cannot be 100% sure. We are also checking stool alpha1 antitrypsin as well as urine protein, both pending.

We have also pending: pneumoccocal and tetanus titers, along wtih mitogen/antigen stim assay.

The possibility of replacing IgG exists if her level continues to fall. However, we are unsure if this would be appropriate given her high volume protein loss. We are considering pharmocokinetics after administering IVIG in attempt to monitor how fast her level will fall should she need it. It is not possible to check an IgG level in her pleural fluid.

Presently, she is not septic, febrile or infectious. She recently underwent bone biopsy of the 8th and 10th ribs after osteopenic and possibly lytic appearing lesions were noted in these ribs on CT. She has a congential absence of the 9th rib. The possibility for malignancy exists as the source of her effusion and we are awaiting pathology.
One other interesting possibility that our other teams have included on the differential is Gorham's Disease, which may explain the changes seen in her ribs and possible a lymphatic proliferation, however this is rare and a diagnosis of exclusion.

We are awaiting the results of an anergy panel  to ensure adequate lymphocyte function in the face of her lymphopenia while waiting for mitogen/antigen studies. Any further assistance you can provide to us on replacement of her IgG in this type of scenario would be helpful. Thank you.


Sean Stanga, M.D.
Fellow, Allergy/Immunology
Children's Mercy Hospital
2401 Gillham Rd
Kansas City, MO 64108
(816)-234-3097

Paul Dowling, MD
Children's Mercy Hospital
________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] On Behalf Of Ballas, Zuhair [zuhair-ballas at uiowa.edu]
Sent: Thursday, December 01, 2011 1:49 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Hypogam following Fontan

Joe
There are also some reports suggesting octreotide might attenuate the loss
Zuhair


From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Church, Joseph
Sent: Thursday, December 01, 2011 10:37 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Hypogam following Fontan

Burcin:  Thank you for your response.  I certainly will keep that approach in mind.  JC

From: pagid-bounces at list.clinimmsoc.org<mailto:pagid-bounces at list.clinimmsoc.org> [mailto:pagid-bounces at list.clinimmsoc.org]<mailto:[mailto:pagid-bounces at list.clinimmsoc.org]> On Behalf Of Burcin Uygungil
Sent: Thursday, December 01, 2011 8:32 AM
To: pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>
Subject: Re: [CIS-PAGID] Hypogam following Fontan

Hi Joe,

I am a mere fellow but I just wrote a review on primary intestinal lymphangiectasia and I think that the same issues apply to this is case of secondary protein losing enteropathy.  In addition to IgG replacement (subq might be better) - you could also try a dietary approach with a high MCT (medium chain triglyceride) diet.  This usually involves a formula so you might need to speak with a dietician to make it more palatable for a 7 year old.  I have seen that it really works in a handful of patients.

Burcin Uygungil
Johns Hopkins Hospital
On Thu, Dec 1, 2011 at 10:17 AM, Church, Joseph <JChurch at chla.usc.edu<mailto:JChurch at chla.usc.edu>> wrote:
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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--
Burcin

"If you want to progress in your life and grow, act not to react"
                                                                       -Yogi Bhajan


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