[CIS PIDD] [cis-pidd] Renal Transplant in CGD

[CIS-PIDD]

The 16 yo daughter of a patient with x-linked CGD (genotype on the father
not done but he is now 47 so I suspect a mild phenotype) now presents in
renal failure due to long standing MPGN. Oxidative burst assay was about
30% of the lower limit of normal and flow analysis showed two cell
populations. Grandmother has similar Lyonization and some clinical
problems. Only the minority population, about 30% of cells, responded to
stimulus. The nephrologist asks if she is a transplant candidate.

There were respiratory tract infections and a mildly low IgG with a normal
IgA and IgM at age five that was felt to be due to protein loss. This
patient has had very inconsistent follow up. She has been seen only five
times since 2009, the last about 18 mo ago so I have limited information
about her recent infections.

Given the diminished phagocytic cell function, will the immunosuppression
necessary for a renal allograph be a high risk for fatal fungal infection?

She was posted to the PAGID List Serve in 2009. See below. At the time,
there were no useful suggestions to explain the reaction to Cellcept. Any
thoughts now?

"Was treated with prednisone for 3 years for MPGN and stopped a year ago.
Cellcept was started. Within 24 hours of starting Cellcept she developed
cervical and submandibular and axillary swollen nodes that were tender with
some fever. Cellcept was stopped and the nodes slowly improved taking one
month to normalize. This occurred three times with the same result. Was
seen by pcp and treated with antibiotics without benefit. Seemed to be
associated with periorbital edema (related to MPGN). Last dose of Cellcept
was one year ago but has had persistent intermittent painful adenopathy."

-- 
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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