[CIS PIDD] [cis-pidd] <no subject>

[CIS-PIDD]

We are certainly looking for virus, in particular vaccine-related VZV given our experience with DOCK 8 and RAG 2 patients given systemic steroids. My experience with more than 50 AT patients is that granulomas are rare and wonder if there is an additional problem in those that develop them and are refractory to treatment.

From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Friday, March 25, 2016 at 4:47 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: Re: [cis-pidd] <no subject>

We have recently identified persistent rubella vaccine in some T cell deficient patients (including AT) with cutaneous granulomas.  One of our patients with that responded to topical steroids and aggressive wound care, the other did not and ultimately died of her ulcers and superinfection.

This work will be presented at the CIS meeting next month and represents a very extensive collaboration with the CDC and many immunologists around the country.





Kate Sullivan, MD PhD
Wallace Chair
Chief of Allergy Immunology
ARC 1216 CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363




On Mar 24, 2016, at 4:06 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:


This is a difficult problem indeed.
I would suggest to pursue all potential routes to prove that in fact there IS an infectious agent involved eg panmicrobial PCR, panfungal PCR (although less likely to be fungal in AT), PCRs for detection of viral genomes,  all necessary cultures etc etc.
Also: did the child receive live vaccines? (Cfr Bodemer C et al) or did he/she have chickenpox (De Somer L et al)?
To what extent is the child immunodeficient at this stage?
Any signs of bone marrow trouble?
Most therapies could do more harm than good but in my opinion after relevant cultures IVIG is a good idea as are empirical broadspectrum antibiotics. Personally would refrain from anti-TNF and opt for systemic steroids as first choice if necessary (short term)... Maybe then thalidomide or another immunomodulating drug?


Isabelle Meyts
UZ Leuven
Belgium




Op 24-mrt.-2016 om 18:39 heeft CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> het volgende geschreven:

I am seeing a 2 year old with with newly discovered ataxia telangiectasia and speeding non-infectious cutaneous granulomas, well described in AT but little success in therapy.
Reports of systemic steroids, high dose topical, anti- TNF not entirely beneficial.
Other thoughts?

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