[PAGID] Eosinophilia in HIES

[Chinen, Javier]

You may know Amy Klion at NIH is an expert in malignant hypereosinophilic syndromes. In the recent past her opinion has been that unless organ damage, close observation is recommended. Especially with a negative bone marrow. She would measure activation markers in eosinophils on a research basis if there is a special concern.
This advice has served me well in a variety of immune dysfunction diagnosis with similar alarming eosinophilia.
Dr. Klion is very helpful.
Javier 
________________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] On Behalf Of Joshi, Avni Y., MBBS [Joshi.Avni at mayo.edu]
Sent: Sunday, May 02, 2010 6:19 PM
To: pagid at list.clinimmsoc.org
Subject: [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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