[CIS PIDD] [cis-pidd] CGD colitis

Wed Jul 30 04:06:51 EDT 2014

Dear Kate, 

this was an extremely educational thread (for me at least!). Should we present it in the Tales at CIS next year?

Mikko

-----Alkuperäinen viesti-----
Lähettäjä: Sullivan, Kathleen [mailto:sullivak at mail.med.upenn.edu] 
Lähetetty: 28. heinäkuuta 2014 22:36
Vastaanottaja: CIS-PIDD
Aihe: Re: [cis-pidd] CGD colitis

Thanks to all!!!!!

I know the Gungor paper but had forgotten they reported the colitis outcomes.

I appreciate all the advice.

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

On Jul 28, 2014, at 1:10 PM, Haddad Elie <elie.haddad at umontreal.ca> wrote:

> Some more precision on steroids for patients with colitis undergoing HSCT: patients received 1mg/kg prednisone until recovery of neutrophils post-HSCT and then prednisone was tapered 0.2 mg/kg/week and steroids were stopped at about 2 months post-HSCT.
> I hope it will help
> All the best
> Elie 
> 
>> On Jul 28, 2014, at 9:45, "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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