[CIS PIDD] [cis-pidd] CGD colitis

[Torgerson, Troy]

Kate,

I would definitely consider transplant in this case.  Our experience has
been similar to Elie¹s with excellent response of the colitis to
transplant.  There is certainly a theoretical risk that active
inflammation in the gut prior to transplant will increase the risk of gut
GvHD but we have not really seen that in our transplanted CGD patients.

Following a pre-transplant course of anti-fungal therapy suggested by
Tayfun Gungor and Reinhard Seger to make sure there are no unpleasant
fungal surprises after conditioning, we use a reduced intensity
Treosulfan/Fludarabine/rATG prep regimen followed by
Methotrexate/Tacrolimus for GvHD prophylaxis.  Engraftment has been
excellent in the CGD patients.

Best,
T

Troy R. Torgerson, MD PhD

Associate Professor of Pediatrics
Divisions of Immunology/Rheumatology
Director, Immunology Diagnostic Lab (IDL)
Co-Director, Non-Malignant Transplant Program
University of Washington and Seattle Children's Hospital

Address:
Seattle Children's Research Institute
1900 9th Ave., C9S-7
Seattle, WA  98101-1304

Phone:  (206) 987-7317
Fax:  (206) 987-7310
Email:  troy.torgerson at seattlechildrens.org

IDL lab:  www.seattlechildrens.org/idl






On 7/28/14, 8:02 AM, "Haddad Elie" <elie.haddad at umontreal.ca> wrote:

>Dear Kate,
>Yes, HSCT clears colitis in most CGD patients. In our hands, we
>experienced this I at least  6 cases, including 3 patients with AR CGD.
>Actually, this outcome is very satisfactory, the patient saying : " I had
>forgotten what it means to have normal stools"...Also, this is associated
>with a growth recuperation (I hope it is the way to say in English)...
>It should cure also the fistula and all the inflammation.
>All the best
>Elie
>
>> On Jul 28, 2014, at 7:11, "Sullivan, Kathleen"
>><sullivak at mail.med.upenn.edu> wrote:
>>
>> I have an unfortunate 14 year old girl with AR CGD who has had
>>essentially untreated colitis for about 10 years.  She has fistulas and
>>tags and extensive vulvar disease.  It is extremely sad.  Her infection
>>pattern has been minimal.
>>
>> Her colitis initially responded to steroids but is now unresponsive to
>>high dose solumedrol and anakinra.  Her disease is sufficiently
>>inflammatory that she has chronic hypoalbuminemia and her nutrition is
>>abysmal even on TPN.  I am looking to hear other folks experience with
>>HSCT in this circumstance.  If it was done in the face of such extensive
>>inflammation- would the HSCT likely clear it or just transform into gut
>>GVHD?
>>
>> Any words of wisdom will be appreciated.
>>
>> Kate
>> Kate Sullivan, MD PhD
>> Wallace Chair of Pediatrics
>> Professor of Pediatrics
>> ARC 1216 Immunology CHOP
>> 3615 Civic Center Blvd.
>> Philadelphia, PA 19104
>> (p) 215-590-1697
>> (f) 267-426-0363
>>
>>
>>
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