[CIS PIDD] Possible DOCK8 with complications

[Kleiner, Gary]

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<mailto: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> [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<mailto: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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