[PAGID] update on an Omenn's patient

[Cowan, Mort]

I would sit tight. So far, you've had a good response to the transplant
and you've done no harm.  Have you looked at radiation sensitivity? Mort

 

Morton J. Cowan, M.D.

Professor of Pediatrics

Chief, 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 devoschildrens.org
Sent: Monday, June 08, 2009 12:57 PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] update on an Omenn's patient

 

Hello,

 I had asked the group for input re a patient of mine more than three
months ago. See below:

 

Patient is a10 months WF, constitutionally XY test feminization, who
presented at 6 weeks with severe Coomb's + AIHA. She then presented on
Dec 24/2008 with PCP pneumonia despite Pentamidine Px which was treated
successfully with Bactrim. She started having a skin rash and diarrhea
with FTT.ALC was 417, CD19=15, CD3= 94, CD4= 38, CD8= 46 and CD16/56=
304. Low IgG and high IgE and eosinophilia, HIV is negative. Mitogen
stimulation was very low at 2-3% of NC (maximal cpm of ~4000 on a
background of 130 for PHA/ ConA). PNP is unlikely with NL uric acid and
ADA-B level was NL. Genetic testing is negative for RAG1/2, JAK-3,
Artemis, IL2RG, IL7RA. Because of this and a positive Rhino in BAL, we
did an unconditioned MSD BMT 3 months ago. Initially we thought she was
engrafting but later on that proved to be likely only peripheral
expansion of donor T cells. The STR is only 7%  donor which is mainly T
cells (55-65%).  Recent CD3=437/Ml, CD4= 246 and CD8= 205. TREC = still
undetectable at <78. CD4 RTE=11.8% and absolute CD4 RTE= 29. CD8 RTE=
0.1 % and absolute CD8 RTE= 0.2 cells. With this low thymic output 3
months post BMT where do I go from here? She has done relatively better
clinically tolerating PEG feeds ( had major dysmotility), less
aspirations after Nissen, almost doubled her weight to 6.5 Kg, better
diarrhea, no rash ( on CSA/steroids), now negative for Rhino but
repeatedly positive for metapneumovirus with only mild respiratory
symptoms.

The questions are: Would she still engraft this late? Likely not. Then
should we move to a myeloablative BMT from the same sister donor? When?
Do we have to wait for metapneumovirus to disappear if it ever will
without good T cell function?

Thanks for your help

 

Aly Mageed, MD, MBA

Division Chief, Pediatric Blood & Marrow Transplant Program

Director, Stem Cell Engineering Laboratory

Helen DeVos Children's Hospital, Spectrum Health

Associate Professor of Pediatrics, Michigan State University

Grand Rapids, MI 

(616)-391-3962

aly.mageed at spectrum-health.org <mailto:aly.mageed at spectrum-health.org> 

 

 

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