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

[CIS-PIDD]

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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