[CIS PIDD] [cis-pidd] Renal transplant in CGD patient with distant h/o aspergillus pneumonia with osteomyelitis

[CIS-PIDD]

Dear Howard,
very interesting questions.
Regarding the IFN, I understand the transplanters and there are many places in the world where CGD patients do not receive IFN as a prophylaxis. On the other hand, you are right, the immunosuppression required for the renal transplantation will increase the rick of infection…but I would do the choice of stopping IFN.
This being said, does the patient have, by chance, an HLA identical related donor ? One could envision a two step strategy with a BMT first (to fix the CGD) and then a renal transplantation with the same donor, with the objective of ending all this without any immunosuppression on the long term. There are some risks, but it may be worth considering this strategy.
If there is no HLA identical related donor (who would accept to give some bone marrow and a kidney…), one can also discuss first a BMT with a MUD before doing the renal transplantation, but here I would say that the risks of a BMT with a MUD at 50 years in order to « just » being able to give an immunosuppression for a renal transplantation is maybe too high.
Would love to hear the opinions from others.
All the best
Elie Haddad

Elie Haddad, MD, PhD,
Professor of Pediatrics, University of Montreal,
Head, Pediatric Immunology and Rheumatology Division,
CHU Sainte-Justine, 3175 Cote Sainte-Catherine
Montreal, QC, H3T 1C5, Canada
Ph: 1 514 345 4713
fax: 1 514 345 4897
e-mail: elie.haddad at umontreal.ca





> Le 2016-12-21 à 11:45, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> a écrit :
> 
> I have a 50 y/o  pt with autosomal recessive CGD, who had aspergillus pneumonia  (dx of aspergillus and then CGD were made only after she had resection of RUL for a persistent pneumonia).  The infection spread to contiguous ribs and vertebrae many, many years ago, despite amphotericin doses that compromised her kidney function.  At just that time, both itraconazole and IFN-gamma were IND’s , and we started both with a long term cure.  She has been maintained on both drugs for probably 20+ yrs, interrupted only briefly for 2 pregnancies.
>  
> She has chronic kidney disease, and is now on the renal transplant list.  The transplant team is not happy with keeping her on IFN, as they think it may increase the rejection risk.  I am very nervous about stopping it, especially in the face of the T-cell immunosuppression that she will need.
>  
> Do any of you have experience with solid organ transplants in CGD?  Any other words of wisdom?
>  
> Howard
> Howard M. Lederman, M.D., Ph.D. 
> Professor of Pediatrics, Medicine and Pathology 
> Division of Pediatric Allergy and Immunology 
> Johns Hopkins Hospital - CMSC 1102 
> 600 N. Wolfe Street 
> Baltimore, MD 21287-3923 
> Phone: 410-955-5883 
> Fax: 410-955-0229 
> Email: Hlederm1 at jhmi.edu <https://mobile.johnshopkins.edu/OWA/redir.aspx?C=02aee18ab96e42ab8cb61c09ecb79487&URL=mailto%3aHlederm1%40jhem.jhmi.edu> 
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