[PAGID] Complete DiGeorge options

[Chinen, Javier]

Just to remind us that the experience unmanipulated BMT for complete DGS is limited to a handful of individual cases reported, and thought to be actually a transplant of committed T cell progenitors (lack of naive cells, similar results with 'transplant' of PBMC),  and also limted to availability of HLA identical donor. 3 unpublished cases of HLA haploidentical BMT did not survive (per Dr. Buckley's BMT chapter on Rich's book 2nd ed)
Dr. Markert's experience with thymus transplant at Duke is of 44 patients tranplanted (HLA haploidentical), with a survival of 75% (33%) and documentation of presence naive T cells and restoration of T cell repertoire, as per her las report in Blood.

Javier

 



Message: 1
Date: Fri, 9 Feb 2007 14:02:18 -0600
From: James Moy <jmoy at rush.edu>
Subject: Re: [PAGID] DiGeorge and GVHD
To: pagid at list.clinimmsoc.org
Message-ID: <B4F76B9C-FC6F-4C51-AB95-E108E47A3B9E at rush.edu>
Content-Type: text/plain; charset="us-ascii"

Thank you.  We will begin communicating with transplantation centers 
immediately.
On Feb 9, 2007, at 9:06 AM, Kathleen E. Sullivan wrote:


> As you well know, the survival for SCID patients with transfusion 

> associated GVHD is extremely poor and the mechanism is roughly the 

> same here.  We did have one SCID survivor after transfusion 

> associated GVHD.  The strategy was to take him for transplant and  

> fully ablate as soon as possible.  During the brief interval prior 

> to transplant we had him on very sturdy immunosuppression.  These 

> days, I might think about Campath as an option.

>

> kate

>

> Kathleen E. Sullivan MD PhD

> Chief, Division of Allergy and Immunology

> Associate Professor of Pediatrics

> The Children's Hospital of Philadelphia

> (p) 215-590-1697

> (f) 267-426-0363

>

>

> On Feb 9, 2007, at 9:51 AM, James Moy wrote:

>

>> Unfortunately, most likely transfusion related.  Our A/I service 

>> was consulted after the initial presentation of GVHD.

>>

>> James N. Moy, M.D.

>> Chief,

>> Division of Allergy and Immunology

>> Department of Pediatrics

>> Stroger Hospital of Cook County

>>

>> Associate Professor,

>> Director,

>> Section of Allergy and Immunology

>> Department of Immunology/Microbiology

>> Rush Medical College

>>

>> On Feb 8, 2007, at 12:27 PM, Kathleen E. Sullivan wrote:

>>

>>> As in maternal GVHD?

>>>

>>> Kathleen E. Sullivan MD PhD

>>> Chief, Division of Allergy and Immunology

>>> Associate Professor of Pediatrics

>>> The Children's Hospital of Philadelphia

>>> (p) 215-590-1697

>>> (f) 267-426-0363

>>>

>>>

>>> On Feb 8, 2007, at 8:19 AM, James Moy wrote:

>>>

>>>> What are the treatment options for a 6 week old with DiGeorge 

>>>> and GVHD with skin and liver involvement?

>>>>

>>>> James Moy, M.D.

>>>> Stroger Hospital of Cook County

>>>> Chicago

>>>

>>

>


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Message: 2
Date: Fri, 9 Feb 2007 18:10:45 -0600
From: "K. Scott Baker" <baker084 at umn.edu>
Subject: Re: [PAGID] DiGeorge and GVHD
To: <pagid at list.clinimmsoc.org>
Message-ID: <004601c74ca7$e9fcb290$2f055486 at epibakers>
Content-Type: text/plain; charset="us-ascii"

Certainly if a well matched donor were available a reduced intensity
conditioning regimen may be sufficient in this situation and something to
consider.   I would agree that a transplant will be necessary.



K. Scott Baker, MD, MS

Pediatric Blood and Marrow Transplant Program

University of Minnesota

420 Delaware St. SE, Mayo Bldg. Room D557

Mayo Mail Code 484

Minneapolis, MN 55455

612.625.4952  FAX 612.626.1434

baker084 at tc.umn.edu

  _____ 

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of James Moy
Sent: Friday, February 09, 2007 2:02 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] DiGeorge and GVHD



Thank you. We will begin communicating with transplantation centers
immediately.

On Feb 9, 2007, at 9:06 AM, Kathleen E. Sullivan wrote:





As you well know, the survival for SCID patients with transfusion associated
GVHD is extremely poor and the mechanism is roughly the same here. We did
have one SCID survivor after transfusion associated GVHD. The strategy was
to take him for transplant and fully ablate as soon as possible. During the
brief interval prior to transplant we had him on very sturdy
immunosuppression. These days, I might think about Campath as an option.



kate



Kathleen E. Sullivan MD PhD

Chief, Division of Allergy and Immunology

Associate Professor of Pediatrics

The Children's Hospital of Philadelphia

(p) 215-590-1697

(f) 267-426-0363







On Feb 9, 2007, at 9:51 AM, James Moy wrote:





Unfortunately, most likely transfusion related. Our A/I service was
consulted after the initial presentation of GVHD.



James N. Moy, M.D.

Chief,

Division of Allergy and Immunology

Department of Pediatrics

Stroger Hospital of Cook County



Associate Professor,

Director,

Section of Allergy and Immunology

Department of Immunology/Microbiology

Rush Medical College



On Feb 8, 2007, at 12:27 PM, Kathleen E. Sullivan wrote:





As in maternal GVHD?



Kathleen E. Sullivan MD PhD

Chief, Division of Allergy and Immunology

Associate Professor of Pediatrics

The Children's Hospital of Philadelphia

(p) 215-590-1697

(f) 267-426-0363







On Feb 8, 2007, at 8:19 AM, James Moy wrote:





What are the treatment options for a 6 week old with DiGeorge and GVHD with
skin and liver involvement?



James Moy, M.D.

Stroger Hospital of Cook County

Chicago









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