[PAGID] Eosinophilia in HIES

[Church, Joseph]

STAT3 mutations may present with neonatal dermatitis, mild elevations of IgE and Pneumocystis jiroveci pneumonia (most recently Ong et al Ann Allergy Asthma Immunol 2010;104:93-4).

 

We have found that meticulous attention to TMP-SMX prophylaxis and skin hygiene in HIES infants has generally limited the extent of skin involvement, IgE elevation and eosinophilia.

 

Rarely, we have used systemic steroids for brief periods in patients with severe inflammatory skin disease.

 

Joe Church

 

 

________________________________

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Freeman, Alexandra (NIH/NIAID) [E]
Sent: Monday, May 03, 2010 6:21 AM
To: 'pagid at list.clinimmsoc.org'
Subject: Re: [PAGID] Eosinophilia in HIES

 

Hi, We have had a few patients with HIES due to STAT3 mutations develop PCP as infants, without prior steroid treatment. We wrote a case series about them a few years ago and have seen one infant since that presented also with PCP as the first pneumonia. If you look at the report, though, it was prior to identification of STAT3 as the molecular etiology-subsequently, mutations were found in all of the pts report except the two sisters with more mild PCP presentations when school-age (ithey have DOCK8 mutations). So, it's not common, but can occur. I would not add immunosuppressants for the purpose of decreasing eosinophilia-we have not needed specific treatment for this in our stat3 patients.
Alexandra

 

________________________________

From: Renner, Eleonore Dr. <Ellen.Renner at med.uni-muenchen.de> 
To: 'pagid at list.clinimmsoc.org' <pagid at list.clinimmsoc.org> 
Sent: Mon May 03 04:09:42 2010
Subject: Re: [PAGID] Eosinophilia in HIES 

Looks like my mentor in Munich was faster. I was just typing a similar question:

 

Hi,

 

Maybe stupid and perhaps already checked BUT is that not also a possible history for OMENN despite the hotspot STAT3 mutation? Is the PCP pneumonia due to high dose steroids? Or does the kid have reduced immunoglobulines, lymphopenia in subsets, or lack of lymphocyte stimulation function to mitogen or antigen as seen in SCID? We have not seen PCP in HIES as a classic infection as stated by Professor Belohradsky but do see eosinophilia. Despite AR-HIES patient with DOCK8 mutations with massive esoniophilia and CNS involvement (perhaps as a result of eosinophilia) STAT3-HIES patient don't need particular treatment for high serum-IgE or eosinophila as to our experience. But there may be different opinion on that? 

 

Kind regards, 

Ellen Renner

 

 

Priv.-Doz. Dr. med. Ellen D Renner

 

Kinderklinik und Kinderpoliklinik 

im Dr. von Haunerschen Kinderspital

Ludwig Maximilians Universität

 

Lindwurmstr. 4

D-80337 München

 

Germany

 

Phone: **49-(0)89-5160-3188

            **49-(0)89-5160-3157 (Labor)

                **49-(0)89-5160-2811 (Pforte)

Fax:         **49-(0)89-5160-7759

 

Ellen.Renner at med.uni-muenchen.de

 

 

________________________________

Von: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] Im Auftrag von Belohradsky, Bernd Prof.Dr.
Gesendet: Montag, 3. Mai 2010 09:50
An: 'pagid at list.clinimmsoc.org'
Betreff: Re: [PAGID] Eosinophilia in HIES

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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