[CIS-PAGID] non conditioned BMT in a SCIDs case

[Cowan, Mort]

It seems like you have a desirable outcome in terms of T and B cell recovery and apparently clinically well. We've followed similar children with this degree of T cell immunity for many years and they are thriving. If she responds normally to killed vaccines with good antibody AND a normal T cell proliferative response to tetanus, then you should go ahead with live viral vaccines. We often start with varicella since if there is a problem you can always treat with acyclovir.

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 Aly.Mageed at helendevoschildrens.org
Sent: Friday, May 11, 2012 10:15 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] non conditioned BMT in a SCIDs case

What does the group think of a 4YO with a SCIDs/Omenn's with an unidentified molecular basis who was transplanted from a full sib without conditioning more than 3 years ago? She is doing well clinically without serious/unusual infections. However, she has stalled in donor chimerism to only 10-14%, her lymphocytes are 96% donor (likely 100% if it were very pure without myeloid contamination). Yet, she continues with CD3/4/8 lymphopenia (CD3 is 400-1000), mostly 400-500. Most recent CD4 were 250. CD4RTE 27%, TREC is low at 280-350 (600-700/million CD3), CD19 last was 300. She is surprisingly maintaining her own IgG level 400-500. Despite the lymphopenia, PHA is good at 60-70% functionally. She responded well to non live vaccines with protective levels against Hep B, DPT and prevnar.
So the Qs are:

1.    Is this good enough at this stage since she is clinically well?

2.    Could she get live vaccines?

3.    Or, Does she need a graft boost?....
Thanks

Aly Mageed, MD, MBA
Pediatric Blood & Marrow Transplant
Helen DeVos Children's Hospital
aly.mageed at helendevoschildrens.org<mailto:aly.mageed at helendevoschildrens.org>




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