[PAGID] BMT and Omenn

[Christine Seroogy]

Dear Colleagues:

I am writing to inquire about bone marrow transplantation for Omenn  
Syndrome.  We have a 4 m/o female (born to consanguinous Amish  
parents) who presented with overwhelming sepsis (grew HIB,  
unvaccinated community.)  She has a clinical history of term birth  
and skin rash (waxing and waning erythroderma/eczematoid; skin bx  
read as seborrheic dermatitis, no cellular infiltrate) since 10 days  
of age, family history of female sib died first month of life with  
pseudomonas sepsis and skin rash since birth (no immune evaluation  
but review of CBC showed lymphopenia and eosinophilia.)

Laboratory findings on this infant demonstrate no B cells, +NK cells,  
and T cell numbers that are all over but persistent CD4 predominance  
and mostly RO and 60% DR+.  She has eosinophilia and IgE 1310.

She is recovering from her sepsis syndrome and here is my question.   
She has a 2y/o sib in good health.  Given the cultural issues, I  
wanted to be a little more precise about BMT in the setting of Omenn  
Syndrome.  The sib has not been HLA typed yet (and I am not sure the  
family will agree to this yet.)  If the sib is a match, it seems the  
success rate is fairly high.  If the sib is not a match, haplo-BMT  
data I could find does not look very favorable and I was wondering  
about matched-unrelated cord blood statistics.

I welcome any input and comments.  Thank you!  Chris





Chris Seroogy, M.D.
Assistant Professor
Dept. of Pediatrics
Mail:  H4/474 CSC, Mailstop 4108
Shipping:  H4/431 CSC, Mailstop 4108
600 Highland Ave.
Madison, WI  53792
phone: 608- 263-2652
fax: 608-265-0164

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