[PAGID] Eosinophilia in HIES

[Klion, Amy (NIH/NIAID) [E]]

Dear All,
I thought that I should clarify my position on treatment. In a completely asymptomatic patient with eosinophilia and no evidence of T cell clonality or CEL, I do not treat the numbers. Everything changes if the patient is symptomatic or has evidence of end organ involvement. In a situation such as the child described below, whether or not to treat would depend on the severity of the rash and whether the biopsy showed eosinophilic infiltration. If steroid therapy was ultimately necessary, we would try to determine the lowest effective dose. It is difficult for me to comment more specifically without more information. 
Amy


Amy Klion, MD
Head, Eosinophil Pathology Unit
Laboratory of Parasitic Diseases
National Institutes of Health
Bldg 50, Rm 6351
Bethesda, MD 20892
(301) 435-8903
(301) 451-2029 (FAX)
aklion at nih.gov

-----Original Message-----
From: Fleisher, Thomas (NIH/CC/DLM) [E] 
Sent: Wednesday, May 05, 2010 2:43 PM
To: Klion, Amy (NIH/NIAID) [E]
Subject: FW: Eosinophilia in HIES

Amy,
You may or may not wish to comment regarding this case to Dr. Joshi but since your name was noted by Javier I thought I would pass this on to you. She is a very pleasant doc training in A&I at Mayo.
Tom

-----Original Message-----
From: Chinen, Javier [mailto:jxchinen at texaschildrens.org]
Sent: Sunday, May 02, 2010 9:22 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] Eosinophilia in HIES

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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