[CIS-PAGID] Heart Tranplant in CVID

[Otto, Hans F Maj USAF AFMC 88 MDOS/SGOM]

Dr Caldwell,
I would agree with the consideration that this patient's
hypogammaglobulemia may be secondary to the cardiomyopathy rather than
two different, rare, unrelated, as yet unidentified genetic defects.
The loss if IgG w/o IgA/M +/- lymphopenia via lymphangiectasia seems
more probable in this scenario.  All diagnoses are suspect until
confirmed, do not let diagnostic inertia lead you astray.  If due to his
cardiomyopathy, a transplant would be EXACTLY what this child needs now.
Perhaps a stool alpha-1-antitrypsin could clarify Ig loss. Here are some
articles to consider, sorry they are older, the only thing I have in my
files on this:  
1.	Wilkinson P, Pinto B, Senior J. Reversible protein-losing
enteropathy with intestinal lymphangiectasia secondary to chronic
constrictive pericarditis.  NEJM 1965; 273:1178-1181
2.	Nelson D, Blaese R, Strober W, et al. Constrictive pericarditis,
intestinal lymphangiectasia, and reversible immunologic deficiency. J
Pediatr. 1975; 86: 548-554

Good luck,
Hans 
//signed//
Hans F Otto, MD, Major, USAF, FACP, Diplomate ABAI
Chief, Allergy/Immunology and Immunizations
Regional Allergy Consultant
88 MDG/SGOMA
4881 Sugar Maple Dr
Wright-Patterson AFB, Ohio 45433
Clinic: 937.257.1684
Fax: 937.257.2284 (Attn: Dr Otto)

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Jason W.
Caldwell
Sent: Wednesday, April 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)