[PAGID] Disseminated mycobacterium

[Chris Seroogy]

Dear Colleagues:

I would like your advice on management of a 19 m/o previously healthy
caucasion girl who presented 4 days ago with thrombocyopenia and anemia.
Her bone marrow biopsy revealed numerous AFB+ organisms.  Her blood grew
mycobacterium and pneumococcus and her stool is growing mycobacterium.
Further identification is pending.  She has tremendous hepatospenomegaly and
high fevers.  Family history is incomplete as mother is adopted and parents
are unlikely related.  She is fully immunized (including live vaccines). ALC
2020, IgG and IgM elevated for age. She is being treated with a "cocktail"
of antimicrobials for mycobacterium per our ID team and vancomycin.  She
remains critically ill.

It seems likely that she has a defect in IFN-g/IL-12 axis.  We will be
sending blood to Steve Holland next week.  In the interim, I would like
opinions about using IFN-g (or perhaps IFN-a if this is a complete IFNR1
defect.)  Have any of you empirically used IFN-g in this setting?  Is there
any downside?  How rapid should improvement be observed if there is a
functioning IFNR?  Thank you for your insights, Chris


Chris Seroogy, M.D.

University of Wisconsin

Assistant Professor

Dept. of Pediatrics

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