[CIS-PAGID] Heart Tranplant in CVID

[Cunningham-Rundles, Charlotte]

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)

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