[CIS PIDD] [cis-pidd] CGD colitis

Mon Jul 28 14:11:12 EDT 2014

Kate,
I cannot but repeat what others already told you. Our experience as being part of Tayun’s paper and also a Swedish paper in Acta Paed comparing the life with and without transplantation, clearly speaks in favor of HSCT.  But  also remember Reinhard Seger’s analysis telling us that those with active inflammation do worst - thus inflammation needs to be controlled as Elie points to - prednisolone 1mg/kg throughout the transplantation and only slowing tapering after SCT has worked for us.

Best
Anders

Anders Fasth, MD, PhD
Professor of Pediatric Immunology, 
Dept of Pediatrics, University of Gothenburg
Address: The Queen Silvia Children’s Hospital,
SE-416 85 Göteborg, Sweden
Tel +46-31-343 5220 (343 4000 switchboard)
Mobile +46-76-050 6117 (work) +46-70-687 5970 (private)
Fax +46-31-707 0694

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Thank you

On 28 jul 2014, at 18:44, Torgerson, Troy <troy.torgerson at seattlechildrens.org> wrote:

> 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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