[CIS PIDD] [cis-pidd] Transplant for Hyper-IgM Syndrome

[CIS-PIDD]

Joe,
Thanks. My thoughts exactly.
Richard

On Tue, Sep 20, 2016 at 7:17 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Richard:
>
>
>
> I am sure there are individuals with more experience than me with this
> disorder.  However, after having a couple of kids die while the family
> contemplated the procedure or were holding out for a 10/10 match when
> ‘only’ a 9/10 was available, I tend to be a bit more aggressive than in the
> past.
>
>
>
> My answer to your question is “transplant when appropriate.”
>
> -       Assume that a transplant will be necessary at some point.
>
> -       Earlier is usually better.
>
> -       Prior to the onset of an opportunistic process is way better.
>
> -       Matched sibling donor is better than MUD is better than haplo.
>
>
>
> I think X-Hyper IgM allows for some deliberation as you can usually buy
> time with IGSC and TMP-SMX.  However, I don’t think it is too early to type
> the family and begin the donor search.
>
>
>
> Joe Church
>
> Children’s Hospital Los Angeles
>
>
>
>
>
>
>
>
>
> *From:* CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
> *Sent:* Tuesday, September 20, 2016 10:53 AM
> *To:* CIS-PIDD
> *Subject:* [cis-pidd] Transplant for Hyper-IgM Syndrome
>
>
>
> Colleagues,
>
> I care for a 7 year old with classic Hyper-IgM Syndrome (single base pain
> substitution g.3715C>A in exon 2 [Genbank EF064753] of CD40L) who has
> virtually never been sick. He was diagnosed early because of an affected
> uncle and has been treated with IGSC and TMP-SMX prophylaxis.
>
>
>
> He has a younger female sib who has not yet been tested. Neither have been
> tissue typed.
>
>
>
> When should this child be transplanted? Would the answer depend on the sib
> matching? If the sib is not a match, what is the current feeling on cord
> blood, MUD or haplo?
>
>
>
> Thanks,
>
> Richard
>
>
>
> --
>
> 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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-- 
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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