[PAGID] update on an Omenn's patient

[Aly.Mageed at devoschildrens.org]

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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