[PAGID] Eosinophilia in HIES

[Joshi, Avni Y., MBBS]

Hello All,

I am mailing to ask you for some input about a patient :

2 month old /M with H/O rash and eosinophilia( AEC: 6700) referred to us
in early Jan. from South Dakota.H/O neonatal rash.

Hypereosinophilia w/up was negative ( with a neg. bone marrow Bx) except
IgE levels which were 721kU/ml.

He was treated with 2mg/kg PO Prednisone for about 3 wks and then with
normalization of Eo count, it was tapered.

While the prednisone was being tapered, he developed PCP pneumonia and
then STAT 3 testing was sent and was found to have STAT3 mutation(
heterozygous for R382Q).

Now he is on daily Bactrim prophylaxis ( both for PCP and staph issues)
and off steroids.

His Eo count is rising again- and we were wondering about re-starting
steroids or considering other steroid sparing regimens.

Thoughts?

 

Thanks,

Avni

Avni Y Joshi, MBBS, M.D.
Fellow in Allergy/Immunology & Pediatric Infectious Diseases 
Instructor in Medicine and Pediatrics
College of Medicine
Phone: 507-293-0865
Secretary: 507-538-0154
Fax: 507-284-0727
E-mail: joshi.avni at mayo.edu
_______________________________
Mayo Clinic
200 First Street SW
Rochester, MN 55905
www.mayoclinic.org <outbind://4/www.mayoclinic.org> 

 

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