[CIS PIDD] Possible DOCK8 with complications

[Church, Joseph]

Thank you all for your comments and suggestions.  

We have an HLA-identical sib so we are hoping to get by with reduced conditioning.

The CNS JC virus is our major concern.  We figure that rapid reconsititution is our patient's best hope.

JC  

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Michael Albert
Sent: Wednesday, July 11, 2012 8:38 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] Possible DOCK8 with complications

I would prefer BuFlu (or if available TreoFlu) over Flu Mel, because you might want a little more myeloablation. Rituximab sounds good.
Actually the JC in the CSF concerns me more than the EBV, but I have no good suggestion what to do about it other than fast immunereconstitution, so I hope you have a well matched donor and will be able to withdraw immunosuppression early.
Michael


> Michael Albert, MD

> Assistant Professor

> Department of Pediatric Hematology/Oncology Head SCT Program Dr. von 

> Haunersches Kinderspital der LMU

> Lindwurmstr.4

> 80337 München

> Germany

> Tel: +49 89 5160 2811

> Fax: +49 89 5160 4719


On Wed, Jul 11, 2012 at 1:00 AM, Kleiner, Gary <GKleiner at mhs.net> wrote:

> Agree with mort

> Flumethiotepa  with a cd34 selected graft and ebv ctl addback may work 

> as well

>

> I would be very reluctant to add csa or fk506 post hsct with the ebv 

> issue if you can wait for ctls Assuming donor is seropos

>

> G

>

> Gary Kleiner MDPhD

>

>

> On Jul 10, 2012, at 5:53 PM, "Cowan, Mort" <mcowan at peds.ucsf.edu> wrote:

>

> Joe,

>

>

>

> It might be worth documenting which cells (T, B or NK) the EBV is 

> residing and also generating EBV specific cytotoxic T cells from donor 

> (if the donor is EBV-seropositive). Catherine Bollard at Baylor has a 

> protocol for doing this. In terms of a protocol, using rituxan is a 

> good idea (assuming it's the B cells that are involved). I'm not sure 

> if a BuFlu or a MelFlu would be better in this case, both are reduced 

> toxicity regimens and both should engraft. Maybe, Mel crosses the BBB 

> a little less well that bu but I'm not sure??

>

>

>

> Mort

>

>

>

> Morton J. Cowan, M.D.

>

> Professor of Pediatrics

>

> Chief, Allergy, Immunology, and Blood and Marrow Transplant Division

>

> UCSF Children's Hospital, Room M659

>

> 505 Parnassus Ave

>

> San Francisco, CA 94143-1278

>

>

>

> Phone: 415-476-2188

>

> FAX: 415-502-4867

>

>

>

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> From: pagid-bounces at list.clinimmsoc.org 

> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Church, Joseph

> Sent: Monday, July 09, 2012 7:21 PM

> To: pagid at list.clinimmsoc.org

> Subject: [CIS PIDD] Possible DOCK8 with complications

>

>

>

> Colleagues:

>

>

>

> We are caring for a 15yo boy from the Middle East.

>

>

>

> He has many features of a DOCK8 mutation (genetic studies are pending) 

> and we are preparing him for BMT from his HLA-identical sibling.

>

>

>

> His major problem is progressive neurologic symptoms, primarily 

> cerebellar, and likely related to his documented:

>

> ·         CNS vasculopathy (dx'd with MRI angiography)

>

> ·         EBV - present in CSF (normal LFTs, no adenopathy or organomegaly).

>

> ·         JC virus - present in CSF.

>

>

>

> Your thoughts regarding the following would be much appreciated:

>

>             1. Conditioning regimen?

>

>             2. Pre-transplant rituximab to reduce EBV?

>

>

>

> Thanks.

>

>

>

> Joe Church

>

> Children's Hospital Los Angeles

>

>

>

>

>

>

>

>

>

>

>

>

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