[CIS PIDD] [cis-pidd] CVID, NRH and HSCT?

[Bleesing, Jacob]

Zachary:

One of our team members is writing up the CCHMC experience of combined (better: sequential) liver and bone marrow transplantation (and will hopefully be submitted for publication within the next couple of weeks).

In our series of 8 (if I kept count correctly), the experience seems to be that the stem cell source does not have to be the same as the liver donor. 90 days may be a good time frame but it does not appear to be written in stone (both shorter and longer likely work - in this case the pathophysiology will likely not return as your patient will be on anti-rejection drugs).

We can discuss this further outside CIS-PIDD.

Jack




________________________________
From: Cowan, Mort [mcowan at peds.ucsf.edu]
Sent: Friday, May 10, 2013 6:24 PM
To: CIS-PIDD
Subject: RE: [cis-pidd] CVID, NRH and HSCT?

Ideally, if the liver and bone marrow donor are the same person (living related?) that would be the best. Years ago we did a transplant on a child with aplastic anemia using Dad as donor. Several years later she developed renal failure from amphotericin toxicity prior to her BMT and received a kidney from Dad. She’s many years out now, off all immunosuppression and married with a child.

We also recently did a haplo (mom) transplant on a child who developed MDS/AML after receiving a cadaver liver transplant. We used highly enriched stem cells from Mom and this child is ~10 months out fully engrafted but still on cellcept and tacrolimis with unmeasurable TAC levels (trying to convince liver team to stop the TAC).

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: Zachary D. Jacobs, MD [mailto:zjacobs.md at gmail.com]
Sent: Friday, May 10, 2013 11:03 AM
To: CIS-PIDD
Subject: [cis-pidd] CVID, NRH and HSCT?

Hello all,

I follow 40 year-old man with CVID, diagnosed about 15 years ago.  For the last several years he has had worsening nodular regenerative hyperplasia associated with his disease, eventually causing portal hypertension, hepatic encephalopathy and TIPS placement.  He is now on the liver transplant list and has actually been given a waiver for this, given his CVID.  Hepatology would like for him to receive a HSCT within 90 days of him receiving his new liver (assuming the transplant happens), to minimize the risk of the pathophysiology repeating itself.

Does anyone have any experience with this?  Would an immunology center in the US like to evaluate him any further for this?

Thanks,

Zach

--
Zachary D. Jacobs, M.D.

The Center for Allergy & Immunology

Saint Luke’s Physician Partners
Medical Plaza II
4330 Wornall, Suite 40
Kansas City, MO 64111

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