[CIS PIDD] [cis-pidd] CVID, NRH and HSCT?

[Claudia Wehr]

Dear all,

in a joint initiative of EBMT "Inborn errors working party" (IEWP) and  
the ESID, we have in 2012 launched a retrospective study to assess the  
outcome and immunological reconstitution of patients with predominant  
antibody deficiency after allogeneic stem cell transplantation  
(StemPAD).
Our study initiative was presented at the ESID meeting in Florence in  
2012 and was
discussed at the EBMT IEWP meeting in April 2012 in Geneva and in  
November 2012 in
Barcelona.

The study is currently recruiting, so far we have collected more than  
20 patients in Europe. We are currently discussing with our colleagues  
from CIBMTR on the possibility of putting the study efforts together.

It is planned to publish the results of the the study this year.  
Concerning your question regarding NRH and HSCT there were so far 2  
adult patients with CVID and NRH who underwent HSCT for lymphoma or  
severe autoimmune phenomena, both died peri-transplant from viral  
infections.

If anyone would like to participate for our study, please contact  
myself (claudia.wehr at uniklinik-freiburg.de), Marta Rizzi  
(marta.rizzi at uniklinik-freiburg.de) or Klaus Warnatz  
(klaus.warnatz at uniklinik-freiburg.de) for mor information. We offer  
1-2 co-authorships per participating center.

With best wishes from Freiburg (Germany)
Claudia Wehr

-- 
University Hospital Freiburg
Centre for Chronic Immunodeficiency (CCI)
Dr. med. Claudia Wehr
Breisacher Str. 117
79104 Freiburg
Germany
Tel: +49-0761-270-71100



Quoting Elie Haddad <elie.haddad at umontreal.ca>:


> Hello,

> Actually we have transplanted one patient who had a CVID and who   

> suffered from a GLILD and an autoimmune granulomatous process that   

> involved the CNS that became eventually resistant to Rituximab. She   

> received a double cord blood transplantation 3 years ago, when she   

> was 16 year old. Her immune reconstitution is not that fantastic,   

> particlularly if one looks at her T cells, with no naive T cells.   

> She is still under immunoglobulins, but does not suffer anymore of   

> her GLILD or of her autoimmune process in CNS. One could say that   

> she is alive and well. Nevertheless, she recently presented with a   

> severe haemolytic autoimmune anemia....and we had to give Rituximab.

> 3 cases have been published in JACI.

> There is presently a project aimed to compil all patients   

> transplanted for CVID that were reported to CIBMTR and this project   

> is leaded by Geoff Cuvelier, from Winnipeg.

> Hope this helps

> Elie

>

> Elie Haddad, MD, PhD,

> Professor of Pediatrics, University of Montreal,

> Head, Pediatric Immunology and Rheumatology Division,

> CHU Sainte-Justine, 3175 Cote Sainte-Catherine

> Montreal, QC, H3T 1C5, Canada

> Ph: 1 514 345 4713

> fax: 1 514 345 4897

> e-mail: elie.haddad at umontreal.ca

>

>

>

>

>

> Le 2013-05-10 à 14:47, Sullivan, Kathleen a écrit :

>

>> I know Elie Haddad has translated a few and we have done a couple   

>> here.  There are small numbers at different locations.  It would be  

>>  great to collect the data and the get the news out.  We were   

>> pleased with the outcome of ours (no NRH though)

>>

>> Kate

>> On May 10, 2013, at 2:42 PM, Riedl, Marc wrote:

>>

>>> We have presented a couple of severely affected CVID patients to   

>>> our Heme-Onc HSCT Committee here over the last several years and   

>>> to date they have been unwilling to move forward due to a lack of   

>>> published literature and established protocols.  I wonder if   

>>> anyone from the NIH could comment as I had one patient that was   

>>> "evaluated" there but reportedly not transplanted in part due to   

>>> lack of a suitable donor.

>>>

>>> Best,

>>>

>>> Marc

>>>

>>> Marc Riedl, M.D., M.S.

>>> Associate Professor of Medicine

>>> Section Head, Clinical Immunology and Allergy

>>> UCLA - David Geffen School of Medicine

>>> 10833 Le Conte Ave, 37-131 CHS

>>> Los Angeles, CA  90095-1680

>>> Tel 310.206.4345  Fax 310.267.0090

>>>

>>>

>>> From: "<Zachary D. Jacobs>", MD   

>>> <zjacobs.md at gmail.com<mailto:zjacobs.md at gmail.com>>

>>> Reply-To: CIS-PIDD   

>>> <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>

>>> Date: Friday, May 10, 2013 11:02 AM

>>> To: CIS-PIDD   

>>> <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>

>>> Subject: [cis-pidd] CVID, NRH and HSCT?

>>>

>>> Hello all,

>>>

>>> I follow 40 year-old man with CVID, diagnosed about 15 years ago.   

>>>  For the last several years he has had worsening nodular   

>>> regenerative hyperplasia associated with his disease, eventually   

>>> causing portal hypertension, hepatic encephalopathy and TIPS   

>>> placement.  He is now on the liver transplant list and has   

>>> actually been given a waiver for this, given his CVID.  Hepatology  

>>>  would like for him to receive a HSCT within 90 days of him   

>>> receiving his new liver (assuming the transplant happens), to   

>>> minimize the risk of the pathophysiology repeating itself.

>>>

>>> Does anyone have any experience with this?  Would an immunology   

>>> center in the US like to evaluate him any further for this?

>>>

>>> Thanks,

>>>

>>> Zach

>>>

>>> --

>>> Zachary D. Jacobs, M.D.

>>>

>>> The Center for Allergy & Immunology

>>>

>>> Saint Luke’s Physician Partners

>>>

>>> Medical Plaza II

>>> 4330 Wornall, Suite 40

>>> Kansas City, MO 64111

>>>

>>> Ph: 816.531.0930

>>> Fax: 816.753.2671

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>>

>> Kate Sullivan, MD PhD

>> Professor of Pediatrics

>> ARC 1216 Immunology CHOP

>> 3615 Civic Center Blvd.

>> Philadelphia, PA 19104

>> (p) 215-590-1697

>> (f) 267-426-0363

>>

>>

>>

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>

>

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