[CIS-PAGID] HSCT in CMC with polyendocrinopathy

[Sorensen, Ricardo]

Richard, can you give us some additional information about the
transplant? How was the patient prepared? Did the transplant take? How
complete was/is reconstitution?

 

Ricardo Sorensen

New Orleans

 

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Richard
Wasserman
Sent: Thursday, July 14, 2011 5:09 PM
To: PAGID
Subject: [CIS-PAGID] HSCT in CMC with polyendocrinopathy

 

In early 1998, a then 15 year-old female who had presented at age two
with oral candidiasis and tetany, received a matched, related transplant
from her 10 year-old brother. At the time of transplant she had
established hypothyroidism, hypoparathyroidism, hypoadrenalism, ovarian
failure and chronic hepatitis that, on biopsy was autoimmune vs drug.
Prior to transplant, oral and especially esophageal candidiasis were
very difficult problems; she had no other infections. It was hoped that
transplant would prevent the development of diabetes. 

Her course has been complicated by an episode of brochiolitis obliterans
about one year post-transplant that responded well to high dose steroids
and gamma globulin, and, more recently, some chest infections. There
have been no new manifestations of autoimmunity. Perhaps this experience
is informative in the context of the current thread.
Richard Wasserman

-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788 <tel:%28972%29%20566-7788> 
Fax (972) 566-8837 <tel:%28972%29%20566-8837> 
Cell (214) 697-7211 <tel:%28214%29%20697-7211> 

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