[CIS PIDD] [cis-pidd] Problems with TIPS in CVID with liver disease?

[CIS-PIDD]

Please be sure to exclude ADA2 deficiency !
(NRH + LGLs)

Best,
Isabelle Meyts
UZ Leuven, Belgium

Van: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Verzonden: donderdag 15 december 2016 15:05
Aan: CIS-PIDD <cis-pidd at lyris.dundee.net>
Onderwerp: VS: AW: [cis-pidd] Problems with TIPS in CVID with liver disease?



We don’t have CVI patients with TIPS. However, I have one CVI/TACI patient with portal hypertension and massive portosystemic collaterals (Caput medusae, varices).  His liver biopsy showed clonal T-LGL lymphocytic infiltration of the liver sinusoids. LGL lymphocytosis of BM and blood.

Any experience/data on this kind of situation?

Greetings

Timi Martelius MD PhD
Inflammation Centre/Infectious Diseases
Helsinki University Hospital
PO Box 348
00029HUS
Helsinki
Finland
timi.martelius at hus.fi<mailto:timi.martelius at hus.fi>

Lähettäjä: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Lähetetty: 14. joulukuuta 2016 0:32
Vastaanottaja: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Kopio: Dr. Simone Brass <simone.brass at uniklinik-freiburg.de<mailto:simone.brass at uniklinik-freiburg.de>>
Aihe: Re: AW: [cis-pidd] Problems with TIPS in CVID with liver disease?

We had one CVID patient who had TIPS placed at about 20 years of age due to nodular regenerative hyperplasia of the liver and MASSIVE ascites.  She developed the ascites while building Habitat for Humanity homes in a developing country.  She had 2 huge workups for infectious and malignant etiologies of disease including liver biopsies and PCR’s/stains for every possible infectious organism that our pediatric and adult ID consultants could dream up but continued to have worsening ascites.  In the end, we were draining about 1 lier of ascites fluid every 1-2 days so that she could breath comfortably.  She underwent TIPS, which resolved the ascites then went on to undergo MUD bone marrow transplant using a minimal intensity conditioning regimen due to progressive pulmonary disease that was resistant to treatment.  She rejected the first graft but was re-transplanted and is alive and doing quite well other than some persistent GvHD.  Would be happy to contribute to the case series – please forward the data collection form when available.

Happy Holidays to all,
T

Troy R. Torgerson, MD PhD

Associate Professor of Pediatrics
Divisions of Immunology/Rheumatology
Director, Immunology Diagnostic Lab (IDL)
Co-Director, Non-Malignant Transplant Program
University of Washington and Seattle Children's Hospital

Address:
Seattle Children's Research Institute
1900 9th Ave., C9S-7
Seattle, WA  98101-1304

Phone:  (206) 987-7317
Fax:  (206) 987-7310
Email:  troy.torgerson at seattlechildrens.org<mailto:troy.torgerson at seattlechildrens.org>

IDL lab:  www.seattlechildrens.org/idl<http://www.seattlechildrens.org/idl>



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: Tuesday, December 13, 2016 at 2:10 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Cc: "Dr. Simone Brass" <simone.brass at uniklinik-freiburg.de<mailto:simone.brass at uniklinik-freiburg.de>>
Subject: AW: [cis-pidd] Problems with TIPS in CVID with liver disease?

Hi Charlotte,
as you know we finally have put together our questionnaire on experience with TIPS in CVID like patients. Sorry for taking so long, but we wanted to test it in our cohort first to make sure our questions are not too complicated.
We have a total of 3 patients with TIPS I am currently aware off. Our gastroenterologists require previous bleeding episode in order to perform TIPS.

One died later on of gram negative sepsis, with known enteropathy and the septic infection possibly favored by TIPS
One patient did well so far
One other patient died also of systemic infection (I don’t have the detailed information at this time).
We did not see clinically relevant encephalopathy in our three patients, but this may have to do also with the remaining liver function which was not too bad in our patients despite severe portal hypertension. I wonder whether we have to worry about gram negative systemic infections in our patients due to underlying enteropathy and disturbed gastrointestinal barriers.

We are very interested in collecting the available clinical data on patients with CVID like disorders and TIPS.
If you are willing to contribute to this survey please contact me or Dr. Brass so we are sending the questionnaire out to you

Thank you

klaus
Prof. Dr. med. Klaus Warnatz

MEDICAL CENTER – UNIVERSITY OF FREIBURG
Center for Chronic Immunodeficiency – CCI
at the Center for Translational CellResearch
Department of Rheumatology and Clinical Immunology

Breisacher Str. 115, 79106 Freiburg, Germany
Tel. +49 761 270 77640 / FAX -77600 / Pager 12-7100
klaus.warnatz at uniklinik-freiburg.de<mailto:klaus.warnatz at 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: Dienstag, 13. Dezember 2016 22:48
An: CIS-PIDD
Betreff: [cis-pidd] Problems with TIPS in CVID with liver disease?

Hi,

We saw a  CVID  patent yesterday with nodular regenerative  hyperplasia  of the liver with portal hypertension and ascites—  no bleed but had varices banded.
Had 2 L fluid removed a few days before.  (TIPS) procedure  (transjugular intrahepatic portosystemic shunt) is being considered.

We have only one CVID patient with a TIPS procedure  and she has been doing well overall but with one episode of encephalopathy in about 6 or more years.

Anyone have other experiences, positive or negative?

Charlotte

Charlotte Cunningham-Rundles, MD, PhD

Departments of Medicine and Pediatrics

The David S Gottesman Professor

The Immunology Institute

Mount Sinai School of Medicine

1425 Madison Avenue

New York, NY 10029

Phone: 212 659 9268

Fax: 212 987 5593

Email: Charlotte.Cunningham-Rundles at mssm.edu<mailto:Charlotte.Cunningham-Rundles at mssm.edu>


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