[CIS-PAGID] HSCT in CMC with polyendocrinopathy

[Richard Wasserman]

Ricardo,
The transplant was done at Duke and I don't recall the conditioning regimen
but I believe that she was ablated fully. The reconstitution was robust and
for ten years she received no gamma globulin or antibiotic/antifungal
prophylaxis. Her B cell numbers have been near the lower limit of normal and
have drifted down over the past five years (84/uL to 32/uL). Her IgG was
always around the lower limit of normal; IgM and IgA were normal. The
pneumococcal response was adequate seven years after transplant but she had
no anti-pneumococcal antibody when reevaluated a year ago. At that time, I
felt the need to restart gamma globulin outweighed my interest in her
response to re-vaccination. *In vitro* response to mitogen and antigen has
been, and continues to be, normal.
Richard

On Thu, Jul 14, 2011 at 5:25 PM, Sorensen, Ricardo <RSoren at lsuhsc.edu>wrote:


>  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

> Fax (972) 566-8837

> Cell (214) 697-7211****

>




-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/mailman/private/pagid/attachments/20110714/95b58451/attachment.htm>