[PAGID] Eosinophilia in HIES

[Belohradsky, Bernd Prof.Dr.]

PCP pneumonia is not typical for autosomal dominant HIES; predisone might have been the cause (as you all know, PCP has been described in infants with West syndrome  under prednisone treatment); Bernd H.Belohradsky (pediatrics,immunology,infectious diseases, Munich,Germany)
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Von: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] Im Auftrag von Joshi, Avni Y., MBBS
Gesendet: Montag, 3. Mai 2010 01:19
An: pagid at list.clinimmsoc.org
Betreff: [PAGID] Eosinophilia in HIES


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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