[CIS PIDD] [cis-pidd] Neutropenia and low interferon gamma production

Thu Sep 4 11:02:38 EDT 2014

you could do a quantiferon TB gold test on him.  the positive control tube will tell you whether he can produce interferon or not.

has he had genetic testing for ELA2 or HAX1?  antigranulocyte antibodies?

Thomas G. Boyce, MD, MPH
Pediatric Infectious Diseases and Immunology
Mayo Clinic
email: boyce.thomas at mayo.edu
phone: 507-255-8464
fax: 507-255-7767

From: Wiens, Lynn A MD [mailto:lawiens at saintfrancis.com]
Sent: Thursday, September 04, 2014 9:40 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] Neutropenia and low interferon gamma production

Initially, he had frequent episodes of cellulitis and one pneumonia. Did culture MRSA with one of the skin lesions.  Cellulitis has greatly improved with treatment.

Lynn A. Wiens, MD
Cell: 918-550-1200
http://tulsaallergynews.com
[cid:image001.png at 01CFC827.5B3ABE90]

From: Kumar, Ashish [mailto:Ashish.Kumar at cchmc.org]
Sent: Thursday, September 04, 2014 09:29
To: CIS-PIDD
Subject: RE:[cis-pidd] Neutropenia and low interferon gamma production

Has he had any serious pyogenic infections or was the neutropenia discovered on CBC? Most children of this age will have benign autoimmune neutropenia for which Neupogen is not really necessary. I am not sure what to make of all the other results in the context of a healthy child.

Ashish Kumar, MD, PhD
Cancer and Blood Diseases Institute
Division of Bone Marrow Transplantation and Immune Deficiency
Cincinnati Children's Hospital Medical Center
http://www.cincinnatichildrens.org/bio/k/ashish-kumar/
http://www.cincinnatichildrens.org/research/divisions/b/bone-marrow/labs/kumar/default/

From: Wiens, Lynn A MD [mailto:lawiens at saintfrancis.com]
Sent: Thursday, September 04, 2014 10:01 AM
To: CIS-PIDD
Subject: [cis-pidd] Neutropenia and low interferon gamma production

I am taking care of a 2 yo male with idiopathic neutropenia doing well on Neupogen.  As part of his work-up, I obtained T-cell function and PHA did upregulate CD 69 but there was no interferon gamma production.  In vitro proliferation to tetanus toxoid, viral mixture, candida was low.  Response to PWM; staph superantigen was normal.

My question is what additional genetic testing is indicated in a boy doing well with neupogen; no additional infections since starting.

Lynn A. Wiens, MD

Warren Clinic

6160 South Yale Avenue  Tulsa, OK 74136

Specializing in the care of Allergy, Asthma, Sinus disease, and Chronic Infections

http://tulsaallergynews.com

Office: 918-495-2636

Cell: 918-550-1200

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