[CIS-PAGID] Heart Tranplant in CVID

[Jason W. Caldwell]

All the thoughts are greatly appreciated.   I should clarify several things.  Diagnosed in 2002 (DOB 1994) with "CVID".  IgG in the 300s on several occasions with no pneumococcal response.  IgA was also low at that time.  Tetanus titer was present prior to being put on replacement, but was decreasing on several repeats.  Prior to being placed on IVIG he has at least multiple pneumonias and his first interaction with the immunology group here was for a severe pneumonia requiring hospitalization and IV antibiotics to clear.  He also had OM and sinus disease reported in the record.  (I have not be able to talk to mom or dad. His social situation is terrible and likely part of the reason for his progressive heart failure, foster care is definitely already on the table.  In 2002 he was placed on IVIG.  When he returned to clinic in 2007 he had been off IVIG for at least a year and his IgG was 298.  He was revaccinated and had no response to tetanus or diphtheria or pneumococcus. His IgA was low at that visit.  Chest imaging in 2002 had shown bronchiectatic changes and a chest film in 2007 demonstrated persistent bronchiectatic changes in left lower lobe.  At that time (2007) and echo was done showing stable mild to moderate LV dysfunction at that time he was on dig and catipril.  For this admission the patient and the parents have admitted to not taking his cardiac meds for some time.  His presentation was respiratory distress with pneumonia.  I questioned the diagnosis of CVID (as I always question the diagnosis reported in the chart) when I saw him a few days ago.  When I was asked to see him he was critically ill with undetectable IgG.  Unfortunately, I cannot make a definitive diagnosis of CVID at this time.  I hope this history helps to clarify his clinical course over the years.  I don't think that his hypogam can be total explained by protein loss secondary to his past history.  Although I do not take for granted a diagnosis reported in a chart, I believe I will still be faced with the question of immune deficiency, since it is the chart, as he is evaluated for transplantation.  

Thanks again for the comments!

Jason Cadlwell

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Routes, John
Sent: Wednesday, April 27, 2011 3:35 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Heart Tranplant in CVID

I agree with Charlotte, highly suspect protein loss and not CVID

Jack Routes




________________________________
From: "Cunningham-Rundles, Charlotte" <charlotte.cunningham-rundles at mssm.edu>
Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Date: Wed, 27 Apr 2011 14:04:26 -0500
To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>, "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Subject: Re: [CIS-PAGID] Heart Tranplant in CVID

Hi,

To me the thing is that with the normal IgA and IgM, one suspects that he has loss of IgG instead of CVID.  With low B cells the memory B cell numbers will also not be very informative either, and unless he has 0.5% or fewer isotype switched memory B cells, and that piece of information does not add a whole lot.  Is he lymphopenic?   If so the mitogen data may not be useful as the lab can't cope with low numbers.    As he is now back on Ig you do not know if he has loss of actual antibody -- that would tell you more than B cell phenotype. the low IgG does not preclude some antibody being found.   I assume he is in heart failure and likely third spacing.    All that comes into play because if the label CVID is put on him, the transplanters will be reluctant.  If he does not really have that label, then he becomes a sick 16 year old that just needs what ever care is needed. The hypo IgG can be addressed while not adding any   CVID concerns.




Charlotte Cunningham-Rundles MD PhD


isotype
-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org on behalf of Jason W. Caldwell
Sent: Wed 4/27/2011 2:18 PM
To: pagid at list.clinimmsoc.org
Subject: [CIS-PAGID] Heart Tranplant in CVID

I have a 16 year old patient in the hospital that has long standing dilated cardiomyopathy since at least the age of 3.  He was diagnosed with CVID by one of my predecessors.  He was diagnosed in 2002.  He was on IVIG in the past, but it looks like he has not been replacement therapy in at least the last three years.  Currently he has an IgG of <200 mg/dl, normal IgA and IgM, and not responses to tetanus or diphtheria.  His T cell numbers are normal but his absolute B cells are 40 cell/mcL.  (He has had low A in the past).  I am waiting on responses to mitgens and antigens and memory B cells.  I have restarted him on replacement Ig. Unfortunately, he was also lost to follow up in the cardiology clinic.  He presented 10 days ago with progressive dilated cardiomyopathy, CHF, and pneumonia.  He has not done well will to this point.  Cardiology now wants to consider him for a heart transplant and I was asked if his CVID would preclude him from a transplant.

My feeling is that it would not be considered an absolute contraindication.  I have found several cases in the literature where solid organ transplant has been done in the setting of CVID.  One was even a heart/lung transplant secondary to complications of CVID.

Does anyone have any wisdom or experiences they could share about solid organ transplantation in the setting of CVID?  Does anyone feel strongly that a heart transplant in this young man not be done?  (Assuming he meets other transplant requirements including having more consistent care)