[CIS PIDD] [cis-pidd] XIAP colitis BMT

[Dr. Carsten Speckmann]

Dear all,

Some of the colitis patients reported by us (Speckmann et al CLIM 2013) have been transplanted in Hamburg (Kai Lehmberg, Ingo Mueller) and Munich (Michael Albert). I asked the two groups for updated follow-up data:

Munich center:
We transplanted two boys, one MUD one haplo. Colitis went away in both and they are doing fine. You may pm me for details. malbert at med.lmu.de

Hamburg Center:
 
We have transplanted 2 patients with XIAP deficiency and inflammatory bowel disease.
- 5 year old boy. Inflammatory bowel disease. So far no HLH. EBV naive. RIC, sibling donor (Treo, Flu, Alemtuzumab). Good outcome, no severe problems. No activity of IBD since, follow up approx. 1,5 years. Stable chimerism.
- 18 year old man, mild HLH at approx. 6 years, afterwards severe IBD. Transplantation for IBD (not for HLH). Same RIC as above, sibling donor, low grade acute GvHD. No activity of IBD afterwards, follow up approx 3 years. Stable chimerism.

We have transplanted 2 more with XIAP deficiency for HLH (no IBD). One died many years ago after myeloablative conditioning (retrospective diagnosis of XIAP def). The other one is fine after RIC. Both are in the Marsh Paper.

Best wishes, Carsten

--
Sent from my phone - please excuse the brevity and possible typos

Dr. Carsten Speckmann
Pediatrician
Group Leader: Benign lymphoproliferative disorders
Pediatric Hematology and Oncology
and Center for Chronic Immunodeficiency
University of Freiburg
Germany
phone: +49 (0)761 270 43010
fax: +49 (0)761 270 45990
www.uniklinik-freiburg.de/cci/studien/alps.html

--
Sent from my phone - please excuse the brevity and possible typos

Dr. Carsten Speckmann
Pediatrician
Group Leader: Benign lymphoproliferative disorders
Pediatric Hematology and Oncology
and Center for Chronic Immunodeficiency
University of Freiburg
Germany
phone: +49 (0)761 270 43010
fax: +49 (0)761 270 45990
www.uniklinik-freiburg.de/cci/studien/alps.html
> Am 07.04.2015 um 13:01 schrieb Sullivan, Kathleen <sullivak at mail.med.upenn.edu>:
> 
> Good morning!
> 
> I found two published cases of XIAP deficiency who presented with colitis who were transplanted. Looks like RIC conditioning is the consensus but I wonder if folks would be willing to share their outcome experiences on this bulletin board specifically for colitis.  I have a teenage Crohn patient just found to have XIAP deficiency.  
> 
> Have people seen full resolution of the colitis post transplant?
> 
> Any unusual complications to watch for?
> 
> Outcome good generally?
> 
> 
> Thanks so much for sharing.  This is a really nice (and pretty savvy) young man who just wants to know what to expect.
> 
> Kate
> Kate Sullivan, MD PhD
> Wallace Chair 
> Chief of Allergy Immunology
> ARC 1216 CHOP
> 3615 Civic Center Blvd.
> Philadelphia, PA 19104
> (p) 215-590-1697
> (f) 267-426-0363
> 
> 
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