[CIS PIDD] [cis-pidd] AT and rubella lesions

Fri Dec 2 09:18:25 EST 2016

Dear Colleagues,

thank you very much for your important considerations and suggestions. I will keep you posted !

Kind regards, Fabian

Von: Ochs, Hans [mailto:hans.ochs at seattlechildrens.org]
Gesendet: Donnerstag, 1. Dezember 2016 01:21
An: CIS-PIDD
Cc: Ochs, Hans (UW-External); Hauck, Fabian Dr. Dr.med.
Betreff: RE: [cis-pidd] AT and rubella lesions

Dear Fabian,

You must have read Kate Sullivan’s paper just published: Perelygina L, Plotkin S, Russo P, Hautala T, Bonilla F, Ochs HD, Joshi A, Routes J, Patel K, Wehr C, Icenogle J, Sullivan KE.

J Allergy Clin Immunol. 2016 Nov;138(5):1436-1439.e11

We had an AT patient with extensive lesions, identified by the pathologist as “Sweets syndrome”. She died of a malignancy at the age of 7 years.

Her immune deficiency was severe, affecting both cellular and humoral immunity. She had pronounced elevation of IgM, low IgG and IgA.

Very poor antibody to phage immunization.

We found out about the presence of Rubella  in the skin lesions years after hear death.

We tried high dose IVIG, steroids locally and for short intervals systemically, antibiotics,

But never gave antivirals (which were not available in limited types in the early 2000th.

Since IVIG does not make it well into the skin, I would now, knowing the etiology, try soaking

a lesion with IgG, e.g. IVIG or Hizentra, using an occlusion wrap, just to see if neutralizing antibodies in

sufficient concentration would make a difference. If improvements, use local treatment for the rest of the lesions,

if possible.

I do not know if any of the known antivirals would work.

HSCT is not the treatment of choice for obvious reasons, but the Munich group has moe experience with NBS

Than anybody else, except perhaps the Moscow group.

The best

hans

Hans D. Ochs, MD, Dr. med
Professor of Pediatrics | Jeffrey Modell Chair of Pediatric Immunology Research
Center for Immunity and Immunotherapies
Seattle Children's Research Institute | University of Washington

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From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, November 29, 2016 10:33 PM
To: CIS-PIDD
Subject: AW: [cis-pidd] C4 deficiency, Cryoglobulinemia and Hypogammaglobulinemia

Dear colleagues,
I am following a seven year old patient with ataxia teleangiectasia and skin granuloma positive for rubella vaccine virus. Granuloma slowly increase in size and significantly contribute to reduced quality of life. Parents are at present not considering hematopoetic stem cell transplantation as a therapeutical option. Does anyone have experience in treating these granulomas with e.g. antiviral drugs or interferons?
Yours sincerely,
Fabian Hauck

Fabian Hauck, MD, PhD

Attending physician / Head Immunodeficiency Unit and Immunological Diagnostics Laboratoy
Pediatrics / Pediatric Hematology and Oncology / Immunology (DGfI)

Dr. von Hauner Children’s Hospital
Klinikum der Universität München
Lindwurmstr. 4, 80337 München
Germany

Tel.: +49 89 4400-53931
Fax: +49 89 4400-53964
E-Mail: fabian.hauck at med.uni-muenchen.de<mailto:fabian.hauck at med.uni-muenchen.de>

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