[CIS PIDD] [cis-pidd] AW: BMT in STAT3-HIES: Yes or better not?

[CIS-PIDD]

I agree with Stephan.
I also think that in adults and adolescents, the adherence to the prophylactic management is an important issue.
In some patients the severeness of the skin disease and possibly treatment resistant pathogens may also trigger the BMT discussion.
Some patients also have severe psychological issues because of the disease which also have to be addressed.
I would caution of life-style transplant...
Best, Bodo

****************************************
Univ.-Prof. Dr. med. B. Grimbacher

Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Breisacherstraße 115, 79106 Freiburg
bodo.grimbacher at uniklinik-freiburg.de
www.uniklinik-freiburg.de/cci

and

Consultant Immunologist
Institute of Immunity & Transplantation
Dept of Immunology
Royal Free Hospital
UNIVERSITY COLLEGE LONDON
Pond Street
London NW3 2QG
b.grimbacher at ucl.ac.uk
www.centreforimmunodeficiency.com

Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Datum: Thursday 23 February 2017 15:25
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Betreff: [cis-pidd] AW: BMT in STAT3-HIES: Yes or better not?

Dear Ellen,

at the moment I would only discuss this step in the case of early progressive lung disease threatening to result in respiratory insufficiency.

Best wishes, St.



UNIVERSITÄTSKLINIKUM FREIBURG
Sektion Pädiatrische Immunologie (CCI)
Zentrum für Kinder- und Jugendmedizin
Prof. Dr. Stephan Ehl
Medizinischer Direktor CCI
Breisacher Straße 115 · 79106 Freiburg
Telefon: +49 (0)761 270-77300
Telefax: +49 (0)761 270-77744
stephan.ehl at uniklinik-freiburg.de<mailto:stephan.ehl at uniklinik-freiburg.de>
www.uniklinik-freiburg.de<http://www.uniklinik-freiburg.de>
www.uniklinik-freiburg.de/cci<http://www.uniklinik-freiburg.de/cci>

Von: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Gesendet: Donnerstag, 23. Februar 2017 14:53
An: CIS-PIDD
Betreff: [cis-pidd] BMT in STAT3-HIES: Yes or better not?


Dear all,

I was contacted by one of our STAT3-HIES families that they received the suggestion to receive BMT and what I think about this.

I have discussed this shortly with Hans Ochs, Alex Freeman, and Steve Holland with inconsistent out-come.

My concerns about all the involvement of STAT3 not hematopoietic system related issues was shared. On the other hand if BMT helps the hematopoietic system it might be also better for the rest.

I am looking very forward to get to know your current opinion about BMT in STAT3-HIES and what your recommend your patients/families.

Thank you a lot.

Best,

Ellen Renner



Ellen D Renner, MD, Dr. med.



Professor of Translational Immunology

Environmental medicine, Klinikum rechts der Isar
Technische Universität München
Munich, Germany

Ellen.Renner at tum.de<mailto:Ellen.Renner at tum.de>






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