[CIS PIDD] Possible DOCK8 with complications

[Michael Albert]

Now that's helpful. Good luck!
Michael



PD Dr. med. Michael Albert

Oberarzt

Abteilung für Pädiatrische Hämatologie/Onkologie

Leiter der Stammzelltransplantation

Dr. von Haunersches Kinderspital der LMU

Lindwurmstr.4

80337 München

Tel: 089 5160 2785

Fax: 089 5160 4719

Am 11.07.2012 um 18:53 schrieb "Church, Joseph" <JChurch at chla.usc.edu>:


> 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

>> 

>> 

>> 

>> **Confidentiality Notice** This email communication and any 

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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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