[CIS PIDD] Possible DOCK8 with complications

[Michael Albert]

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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